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Giant cell tumor of bone

2016-03-23T15:35:01Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
{{SI}}
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}};{{AE}} {{PSK}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. On gross pathology, [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Common complications of giant cell tumor include malignant transformation, recurrence, and metastasis. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

===Prognosis===
*The [[prognosis]] of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

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{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-23T15:32:36Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
{{SI}}
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}};{{AE}} {{PSK}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. On gross pathology, [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor. Common complications of giant cell tumor include malignant transformation, recurrence, and metastasis.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

===Prognosis===
*The [[prognosis]] of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-23T15:30:11Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
{{SI}}
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}};{{AE}} {{PSK}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. On gross pathology, [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor. Common complications of giant cell tumor include malignant transformation, recurrence, and metastasis.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

===Prognosis===
*The [[prognosis]] of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-23T15:05:10Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
{{SI}}
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}};{{AE}} {{PSK}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. On gross pathology, [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor. Common complications of giant cell tumor include malignant transformation, recurrence, and metastasis.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

===Prognosis===
*The [[prognosis]] of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-23T14:50:48Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
{{SI}}
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}};{{AE}} {{PSK}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor. Common complications of giant cell tumor include malignant transformation, recurrence, and metastasis.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

===Prognosis===
*The [[prognosis]] of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T21:00:54Z

Suveenkrishna Pothuru: /* X Ray */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}};{{AE}} {{PSK}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor. Common complications of giant cell tumor include malignant transformation, recurrence, and metastasis.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

===Prognosis===
*The [[prognosis]] of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:59:29Z

Suveenkrishna Pothuru:

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}};{{AE}} {{PSK}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor. Common complications of giant cell tumor include malignant transformation, recurrence, and metastasis.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

===Prognosis===
*The [[prognosis]] of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone . They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:57:25Z

Suveenkrishna Pothuru: /* Prognosis */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor. Common complications of giant cell tumor include malignant transformation, recurrence, and metastasis.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

===Prognosis===
*The [[prognosis]] of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone . They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:56:39Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor. Common complications of giant cell tumor include malignant transformation, recurrence, and metastasis.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone . They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:56:15Z

Suveenkrishna Pothuru: /* Complications */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone . They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:55:04Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone . They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:51:11Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone . They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:45:13Z

Suveenkrishna Pothuru: /* X Ray */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Metaphyseal location and grow to the articular surface of the involved bone
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone . They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:43:21Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor. X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include metaepiphyseal location of mass and grow to the articular surface of the involved bone with narrow zone of transition.<ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone . They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:42:35Z

Suveenkrishna Pothuru: /* X Ray */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Narrow zone of transition: a broader zone of transition is observed in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix [[calcification]]/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone . They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:30:53Z

Suveenkrishna Pothuru: /* X Ray */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
X-ray may be helpful in the diagnosis of giant cell tumor of bone. Findings on x-ray suggestive of giant cell tumor include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:23:59Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone. Physical examination findings will depend on the location of the giant cell tumor. Common physical examination findings of giant cell tumor are localized [[swelling]] and [[tenderness]] at the site of the tumor.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T20:21:34Z

Suveenkrishna Pothuru: /* Physical Examination */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of giant cell tumor during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T19:42:47Z

Suveenkrishna Pothuru: /* Physical Examination */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the giant cell tumor. Most giant cell tumors are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T19:34:20Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion. On microscopic histopathological analysis, prominent and diffuse osteoclastic giant cells and mononuclear cells with frequent mitotic figures are characteristic findings of giant cell tumor of bone.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T19:33:29Z

Suveenkrishna Pothuru: /* Microscopic Pathology */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T19:25:08Z

Suveenkrishna Pothuru: /* Microscopic Pathology */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===

Microscopically they are characterized by prominent and diffuse osteoclastic giant cells and mononuclear cells ( round, oval, or polygonal and may resemble normal histiocytes). Frequent mitotic figures in the mononuclear cells may be seen, especially in pregnant women or those on the oral contraceptive pill (due to increased hormone levels)

*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*Frequent mitotic figures in the mononuclear cells may be observed, especially in pregnant women or those on the [[oral contraceptive pill]] (due to increased hormone levels).

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T19:04:05Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism. Symptoms of giant cell tumor of bone include localized pain, localized swelling, and decreased range of motion.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T18:41:43Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are distal femur, proximal tibia, and distal radius. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T18:29:21Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. Giant cell tumor of bone must be differentiated from [[aneurysmal bone cyst]], [[chondroblastoma]], simple bone cyst, osteoid osteoma, [[osteoblastoma]], [[osteosarcoma]], and [[brown tumor]] of hyperparathyroidism.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

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{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T18:28:53Z

Suveenkrishna Pothuru: /* Differentiating Giant cell tumor of bone from other Diseases */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T16:15:59Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts. The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T16:15:30Z

Suveenkrishna Pothuru: /* Pathophysiology */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone.

==Pathophysiology==
The progression to giant cell tumor of bone usually involves the over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T16:13:24Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref> On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone.

==Pathophysiology==
Giant cell tumours are believed to result from an over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T16:11:33Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>. On gross pathology, hemorrhage, presence of co-existent [[aneurysmal bone cyst]], and [[fibrosis]] are characteristic findings of giant cell tumor of bone.

==Pathophysiology==
Giant cell tumours are believed to result from an over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T16:10:52Z

Suveenkrishna Pothuru: /* Gross Pathology */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

==Pathophysiology==
Giant cell tumours are believed to result from an over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of presence [[fibrosis]].

===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T15:55:25Z

Suveenkrishna Pothuru: /* CT */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

==Pathophysiology==
Giant cell tumours are believed to result from an over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of the bone

2016-03-22T15:43:31Z

Suveenkrishna Pothuru: ←Redirected page to Giant cell tumor of bone

#redirect[[Giant cell tumor of bone]]

Giant cell tumor of bone

2016-03-22T15:42:15Z

Suveenkrishna Pothuru:

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor; Giant cell tumor of the bone

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

==Pathophysiology==
Giant cell tumours are believed to result from an over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T14:54:15Z

Suveenkrishna Pothuru: /* Prevalence */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

==Pathophysiology==
Giant cell tumours are believed to result from an over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T14:53:27Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells). Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors.<ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

==Pathophysiology==
Giant cell tumours are believed to result from an over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T14:39:43Z

Suveenkrishna Pothuru: /* Pathophysiology */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells).

==Pathophysiology==
Giant cell tumours are believed to result from an over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T14:37:19Z

Suveenkrishna Pothuru: /* Pathophysiology */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells).

==Pathophysiology==
Giant cell tumours are believed to result from an over-expression in RANK/RANKL signalling pathway with resultant over-proliferation of osteoclasts
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum.
===Location===
Giant cell tumor of bone typically occur as single lesions. They usually prefers the epiphyses of long bones. Although any bone can be affected, the most common sites are: <ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-22T13:15:41Z

Suveenkrishna Pothuru: /* Pathophysiology */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells).

==Pathophysiology==
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum. Giant cell tumors usually prefers the epiphyses of long bones.<ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
===Location===
Giant cell tumor of bone typically occur as single lesions. Although any bone can be affected, the most common sites are:
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.

==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-18T14:00:31Z

Suveenkrishna Pothuru: /* Overview */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==
Giant cell tumor of bone is a relatively uncommon tumor of the bone. It is characterized by the presence of multinucleated giant cells (osteoclast-like cells).

==Pathophysiology==
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum. Giant cell tumors (GCT) usually prefers the epiphyses of long bones.<ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
===Location===
Giant cell tumor of bone typically occur as single lesions. Although any bone can be affected, the most common sites are:
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.
==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-16T20:23:50Z

Suveenkrishna Pothuru: /* Treatment */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==

==Pathophysiology==
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum. Giant cell tumors (GCT) usually prefers the epiphyses of long bones.<ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
===Location===
Giant cell tumor of bone typically occur as single lesions. Although any bone can be affected, the most common sites are:
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.
==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref> Surgery is the mainstay of treatment for giant cell tumor.

===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-16T20:20:07Z

Suveenkrishna Pothuru: /* Treatment */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==

==Pathophysiology==
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum. Giant cell tumors (GCT) usually prefers the epiphyses of long bones.<ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
===Location===
Giant cell tumor of bone typically occur as single lesions. Although any bone can be affected, the most common sites are:
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.
==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
The treatment of giant cell tumor is directed towards local control without sacrificing joint function.<ref name="PuriAgarwal2007">{{cite journal|last1=Puri|first1=Ajay|last2=Agarwal|first2=Manish|title=Treatment of giant cell tumor of bone: Current concepts|journal=Indian Journal of Orthopaedics|volume=41|issue=2|year=2007|pages=101|issn=0019-5413|doi=10.4103/0019-5413.32039}}</ref>
===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-16T20:17:29Z

Suveenkrishna Pothuru: /* Complications */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==

==Pathophysiology==
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum. Giant cell tumors (GCT) usually prefers the epiphyses of long bones.<ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
===Location===
Giant cell tumor of bone typically occur as single lesions. Although any bone can be affected, the most common sites are:
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.
==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
Common complications of giant cell tumor include:
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.
===Prognosis===
*The prognosis of giant cell tumor is generally excellent.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-16T20:07:40Z

Suveenkrishna Pothuru: /* Differentiating Giant cell tumor of bone from other Diseases */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==

==Pathophysiology==
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum. Giant cell tumors (GCT) usually prefers the epiphyses of long bones.<ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
===Location===
Giant cell tumor of bone typically occur as single lesions. Although any bone can be affected, the most common sites are:
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.
==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
Giant cell tumor of bone must be differentiated from:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-16T15:36:34Z

Suveenkrishna Pothuru:

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''

{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==

==Pathophysiology==
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum. Giant cell tumors (GCT) usually prefers the epiphyses of long bones.<ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
===Location===
Giant cell tumor of bone typically occur as single lesions. Although any bone can be affected, the most common sites are:
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.
==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
A number of tumors have giant cells, but are not true benign giant cell tumors. These include:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-16T13:14:04Z

Suveenkrishna Pothuru: /* Physical Examination */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''
{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==

==Pathophysiology==
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum. Giant cell tumors (GCT) usually prefers the epiphyses of long bones.<ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
===Location===
Giant cell tumor of bone typically occur as single lesions. Although any bone can be affected, the most common sites are:
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.
==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
A number of tumors have giant cells, but are not true benign giant cell tumors. These include:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''

A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell myeloma

2016-03-16T13:01:00Z

Suveenkrishna Pothuru: ←Redirected page to Giant cell tumor of bone

#redirect[[Giant cell tumor of bone]]

Giant cell tumor of bone

2016-03-16T12:58:07Z

Suveenkrishna Pothuru: /* Treatment */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''
{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==

==Pathophysiology==
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum. Giant cell tumors (GCT) usually prefers the epiphyses of long bones.<ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
===Location===
Giant cell tumor of bone typically occur as single lesions. Although any bone can be affected, the most common sites are:
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.
==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
A number of tumors have giant cells, but are not true benign giant cell tumors. These include:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''
A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-16T12:51:47Z

Suveenkrishna Pothuru: /* Risk Factors */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''
{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==

==Pathophysiology==
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum. Giant cell tumors (GCT) usually prefers the epiphyses of long bones.<ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
===Location===
Giant cell tumor of bone typically occur as single lesions. Although any bone can be affected, the most common sites are:
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.
==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Differentiating Giant cell tumor of bone from other Diseases==
A number of tumors have giant cells, but are not true benign giant cell tumors. These include:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''
A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
===Medical Therapy===
===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

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[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Giant cell tumor of bone

2016-03-16T12:51:14Z

Suveenkrishna Pothuru: /* Laboratory Findings */

__NOTOC__
'''For patient information, click [[Giant cell tumor of bone (patient information)|here]]'''
{{CMG}}

{{SK}} Osteoclastoma; Giant cell myeloma; Giant cell tumor

==Overview==

==Pathophysiology==
Giant cell tumor of bone occurs commonly in the distal femur, the proximal tibia, the distal radius and the sacrum. Giant cell tumors (GCT) usually prefers the epiphyses of long bones.<ref name="ShrivastavaNawghare2008">{{cite journal|last1=Shrivastava|first1=Sandeep|last2=Nawghare|first2=Shishir P|last3=Kolwadkar|first3=Yogesh|last4=Singh|first4=Pradeep|title=Giant cell tumour in the diaphysis of radius – a report|journal=Cases Journal|volume=1|issue=1|year=2008|pages=106|issn=1757-1626|doi=10.1186/1757-1626-1-106}}</ref>
===Location===
Giant cell tumor of bone typically occur as single lesions. Although any bone can be affected, the most common sites are:
:*Around the knee: distal femur and proximal tibia: 50-65%
:*Distal radius: 10-12%
:*Sacrum: 4-9%
:*Vertebral body
:*Multiple locations: 1%

===Gross Pathology===
*Macroscopically, giant cell tumors are variable in appearance, depending on amount of [[hemorrhage]], presence of co-existent [[aneurysmal bone cyst]], and degree of [[fibrosis]].
===Microscopic Pathology===
*Giant cell tumor of bone is characterized by the presence of numerous Cathepsin-K producing, CD33 +, CD14 - multinucleated osteoclast-like giant cells and plump spindle-shaped stromal cells that represent the main proliferating cell population.
*The spindle-shaped mononuclear cells are believed to represent the neoplastic population and are characterized at the cytogenetic level by telomeric associations and a peculiar telomere-protecting capping mechanism.
*Areas of regressive change such as necrosis or fibrosis as well as extensive hemorrhage are frequently present.
*It recurs from time to time and rates between 25–50% have been reported. In very rare cases, a malignant change may occur.
==Epidemiology and Demographics==

===Prevalence===
*Giant cell tumor of bone accounts for 4-5% of primary bone tumors and 18.2% of benign bone tumors <ref name="pmid12579271">{{cite journal |author=Gamberi G, Serra M, Ragazzini P, Magagnoli G, Pazzaglia L, Ponticelli F, Ferrari C, Zanasi M, Bertoni F, Picci P, Benassi MS |title=Identification of markers of possible prognostic value in 57 giant cell tumors of bone |journal=[[Oncology Reports]] |volume=10 |issue=2 |pages=351–6 |year=2003 |pmid=12579271 |doi= |url=http://www.spandidos-publications.com/or/10/2/351 |accessdate=2012-01-18}}</ref>.

===Age===
*Giant cell tumor of bone almost invariably (97-99%) occur when the growth plate has closed and are therefore typically observed in early adulthood, with 80% of cases reported between the ages of 20 and 50, with a peak [[incidence]] between 20 and 30.<ref name=radiopaedia>Giant cell tumor of bone.Dr Henry Knipe and Dr Behrang Amini et al.Radiopaedia.org 2015.http://radiopaedia.org/articles/giant-cell-tumour-of-bone.Accessed on March 11, 2016</ref>

===Gender===
*Giant cell tumor of bone is more common in females with the rate of growth enhanced in pregnancy. Female to male ratio 1.5:1.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref>

==Risk Factors==

==Differentiating Giant cell tumor of bone from other Diseases==
A number of tumors have giant cells, but are not true benign giant cell tumors. These include:
*[[Aneurysmal bone cyst]]
*[[Chondroblastoma]]
*Simple bone cyst
*Osteoid osteoma
*[[Osteoblastoma]]
*[[Osteosarcoma]]
*Giant cell reparative granuloma
*[[Brown tumor]] of hyperparathyroidism
*Non-ossifying fibroma
*Giant cell reparative granuloma

==Natural History, Complications and Prognosis==
===Complications===
*Malignant transformation
:*Malignant transformation is far more common in men (M:F of ~3:1)
:*Sarcomatous transformation is observed, especially in [[radiotherapy]] treated inoperable tumors.
*Recurrence
:*Local recurrence rate of giant cell tumor of bone is 10 to 40%.
:*Recurrence rates are higher when the tumor bone arises at a surgical inaccessible location locations such as [[spine]] and [[sacrum]].
*Metastasis
:*Giant cell tumor of bone may occasionally metastasize to vital organs such as the lung.<ref name="MuheremuNiu2014">{{cite journal|last1=Muheremu|first1=Aikeremujiang|last2=Niu|first2=Xiaohui|title=Pulmonary metastasis of giant cell tumor of bones|journal=World Journal of Surgical Oncology|volume=12|issue=1|year=2014|pages=261|issn=1477-7819|doi=10.1186/1477-7819-12-261}}</ref> Hence, this entity has been called benign metastasising giant cell tumor.

==Diagnosis==
===History and Symptoms===
* Patients usually present with pain and limited [[range of motion]] caused by tumor's proximity to the joint space.
* There may be [[swelling]] as well, if the tumor has been growing for a long time.
* Some patients may be asymptomatic until they develop a pathologic [[fracture]] at the site of the tumor.

===Physical Examination===
Physical examination findings will depend on the location of the osteosarcoma. Most osteoosarcomas are located in the long bone of extremities.

'''Extremities'''
A palpable firm non tender or tender mass may be appreciated on physical examination. The assessment of osteosarcoma during physical examination include:
*Size
*Location
*Involvement of adjacent structures (such as peripheral [[nerves]] or [[veins]])
*[[Edema]

===X Ray===
General radiographic features include:
*Narrow zone of transition: a broader zone of transition is seen in more aggressive giant cell tumors.
*No surrounding sclerosis: 80-85%
*Overlying cortex is thinned, expanded or deficient
*Periosteal reaction is only observed in 10-30% of cases
*Soft tissue mass is not infrequent
*Pathological fracture may be present
*No matrix calcification/mineralisation

On [[x-ray]], giant cell tumors (GCTs) have a metaepiphyseal location and grow to the articular surface of the involved bone <ref name="pmid11553835">{{cite journal |author=Murphey MD, Nomikos GC, Flemming DJ, Gannon FH, Temple HT, Kransdorf MJ |title=From the archives of AFIP. Imaging of giant cell tumor and giant cell reparative granuloma of bone: radiologic-pathologic correlation |journal=[[Radiographics : a Review Publication of the Radiological Society of North America, Inc]] |volume=21 |issue=5 |pages=1283–309 |year=2001 |pmid=11553835 |doi= |url=http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=11553835 |accessdate=2012-01-18}}</ref>. They are distinguishable from other bony [[tumor]]s in that GCTs usually have a non-sclerotic and sharply defined border. Because giant cell tumors are known to metastasize, when the diagnosis of giant cell tumor is suspected, a chest x-ray or [[computed tomography|CT]] may be needed.

(Images courtesy of RadsWiki)
<div align="left">
<gallery heights="175" widths="175">
Image:Giant-cell-tumor-001.jpg|Giant cell tumor: Distal part of the femur
Image:Giant-cell-tumor-002.jpg|Giant cell tumor: Distal part of the femur
</gallery>
</div>

===CT===
===MRI===
Typical signal characteristics on MRI of giant cell tumor of bone include:

'''T1''':
:*Low to intermediate solid component
:*Low signal periphery
:*Solid components enhance, helping distinguish giant cell tumor with [[aneurysmal bone cyst]] from pure aneurysmal bone cyst
:*Some enhancement may also be observed in adjacent [[bone marrow]]
'''T2''':
:*Heterogenous high signal with areas of low signal intensity (variable) due to haemosiderin or fibrosis
:*If an [[aneurysmal bone cyst]] component present, then fluid-fluid levels can be observed
:*High signal in adjacent bone marrow thought to represent inflammatory [[edema]]
'''T1 C+ (Gd)''':
:*Solid components will enhance, helping differentiate from aneurysmal bone cyst
===Scintigraphy: Bone Scan===
*Most giant cell tumors demonstrate increased uptake on delayed images, especially around the periphery, with a central photopenic region (doughnut sign).
*Increased blood pool activity is also observed, and can be observed in adjacent bones due to generalised regional hyperaemia.

==Treatment==
===Medical Therapy===
===Surgery===
*Classically, treatment is with curettage and packing with bone chips or polymethylmethacrylate (PMMA).
*Local recurrence is from the periphery of the lesion and has historically occurred in up to 40-60% of cases.
*Newer intraoperative adjuncts such as thermal or chemical treatment of the resection margins have lowered the recurrence rate to 2.5-10%.
*Wide local excision is associated with a lower recurrence rate, but has greater morbidity.

==References==
{{reflist|2}}

{{WikiDoc Help Menu}}
{{WikiDoc Sources}}

[[Category:Disease]]
[[Category:Types of cancer]]
[[Category:Oncology]]
[[Category:Orthopedics]]