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{{CMG}} {{AE}} {{MV}}
{{CMG}} {{AE}} {{MV}}
   
   
{{SK}} Synonym 1; Synonym 2; Synonym 3
{{SK}} Malignant synovioma
   
   
==Overview==
==Overview==


Synovial sarcoma (also known as [[malignant]] synovioma) is a very rare form of [[soft tissue]] [[sarcoma]], which usually occurs near the [[Joint|joints]] in upper and lower [[Limb (anatomy)|extremities]]. Synovial sarcoma was first discovered by Pack and Tabah in 1955. Synovial sarcoma may be classified according to histopathological findings into 4 sub-types: biphasic, monophasic [[fibrous]] (most common), monophasic [[Epithelium|epithelial]], and poorly differentiated. The [[pathogenesis]] of synovial sarcoma is characterized by the dysregulation of [[gene expression]] of SYT-SSX [[gene]]. The most common locations for the occurrence of synovial sarcoma include [[Knee]], adjacent to large [[Joint|joints]], and [[Popliteal fossa]]. The SYT-SSX fusion [[gene]] (located in [[chromosome]] 18) has been associated with the development of synovial sarcoma. There are no established causes for synovial sarcoma. Synovial sarcoma must be differentiated from other [[Disease|diseases]] that cause [[Arthralgia|joint pain]], mass growth, and limited [[range of motion]], such as [[Malignant fibrous histiocytoma|malignant fibrous histiocytoma (MFH)]]-[[fibrosarcoma]], [[Liposarcoma]], [[Osteosarcoma]], and [[Chondrosarcoma]]. The [[prevalence]] of synovial sarcoma remains unknown. Synovial sarcomas account for 2.5 - 10% of all [[soft tissue]] sarcomas. Synovial sarcoma is more commonly observed among [[Patient|patients]] aged 15 - 40 years old. There is no racial predilection for synovial sarcoma. There are no known [[Risk factor|risk factors]] associated with the development of synovial sarcoma. There is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for synovial sarcoma. The majority of [[Patient|patients]] with synovial sarcoma remain [[asymptomatic]] for years. If left untreated, [[Patient|patients]] with synovial sarcoma may progress to develop [[Metastasis|metastases]]. [[Prognosis]] is generally poor, and the [[median]] survival rate of [[Patient|patients]] with synovial sarcoma is approximately 35% to 60%. The [[diagnosis]] of synovial sarcoma is typically made based on [[histology]] and is confirmed by the presence of t(X;18). There are no specific [[Medical laboratory|laboratory]] findings associated with synovial sarcoma. [[Patient|Patients]] with synovial sarcoma usually are well-appearing. There are no specific [[Medical laboratory|laboratory]] findings associated with synovial sarcoma. There are no [[The electrocardiogram|ECG]] findings associated with synovial sarcoma. Plain [[X-rays|x-ray]] can be normal unless the [[tumor]] is large in size or has [[Dystrophic calcification|dystrophic calcifications]]. On [[ultrasound]], characteristic findings of synovial sarcoma include heterogeneity and hypo-echoic mass. On [[Computed tomography|CT scan]], characteristic findings of synovial sarcoma include [[Soft tissue]] mass, [[Calcification|calcifications]], and [[Heterogeneous]] [[density]] and enhancement. [[Magnetic resonance imaging|MRI]] is the [[imaging]] modality of choice for synovial sarcoma. [[Medicine|Medical]] [[therapy]] include [[Doxorubicin]], [[ifosfamide]], and [[gemcitabine]]. [[Surgery]] is the mainstay of [[therapy]]. [[Surgery|Surgical]] [[resection]] in conjunction with [[chemotherapy]] or [[Radiation therapy|radiation]] is the most common approach to the treatment of synovial sarcoma. There are no established measures for the [[Prevention (medical)|prevention]] of synovial sarcoma.


==Historical Perspective==
==Historical Perspective==
*Synovial sarcoma was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
*Synovial sarcoma was first discovered by Pack and Tabah in 1955.<ref name="pmid19865558">{{cite journal |vauthors=Gomatos IP, Alevizos L, Kafiri G, Bramis J, Leandros E |title=Management of a small incidentally discovered retroperitoneal synovial sarcoma |journal=Can J Surg |volume=52 |issue=5 |pages=E199–200 |year=2009 |pmid=19865558 |pmc=2769101 |doi= |url=}}</ref>
*In [year], [gene] mutations were first identified in the pathogenesis of synovial sarcoma.
 
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose synovial sarcoma.
==Classification==
==Classification==
*Synovial sarcoma may be classified according to [classification method] into [number] subtypes/groups:
*Synovial sarcoma may be [[Classification|classified]] according to [[Histopathology|histopathological]] findings into 4 sub-types:<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
:*[group1]
:*Biphasic
:*[group2]
:*Monophasic [[fibrous]] (most common)
:*[group3]
:*Monophasic [[Epithelium|epithelial]]
*Other variants of synovial sarcoma include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
:*Poorly differentiated
 
==Pathophysiology==
==Pathophysiology==
*The pathogenesis of synovial sarcoma is characterized by [feature1], [feature2], and [feature3].
*The [[pathogenesis]] of synovial sarcoma is characterized by the dysregulation of [[gene expression]] of SYT-SSX [[gene]].<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
*The [gene name] gene/Mutation in [gene name] has been associated with the development of synovial sarcoma, involving the [molecular pathway] pathway.
*The most common locations for the occurrence of synovial sarcoma include:<ref name="pmid19865558">{{cite journal |vauthors=Gomatos IP, Alevizos L, Kafiri G, Bramis J, Leandros E |title=Management of a small incidentally discovered retroperitoneal synovial sarcoma |journal=Can J Surg |volume=52 |issue=5 |pages=E199–200 |year=2009 |pmid=19865558 |pmc=2769101 |doi= |url=}}</ref>
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of synovial sarcoma.
:*[[Knee]]
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of synovial sarcoma.
:*Adjacent to large [[Joint|joints]]
:*[[Popliteal fossa]]
*The SYT-SSX fusion [[gene]] (located in [[chromosome]] 18) has been associated with the development of synovial sarcoma.  
*On [[gross pathology]], characteristic findings of synovial sarcoma include:
:*Solid often [[Lobule|lobulated]]
:*Grey-yellow
:*Pushing border to ill-defined border
*On [[microscopic]] [[Histopathology|histopathological]] [[analysis]], characteristic findings of synovial sarcoma include:<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
:*Non-specific appearances
:*Well or poorly defined [[heterogeneous]] masses
:*Frequent areas of [[Bleeding|hemorrhage]]
:*[[Necrosis]]
   
   
==Causes==
==Causes==
* Synovial sarcoma may be caused by either [cause1], [cause2], or [cause3].
* Synovial sarcoma is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
* There are no established causes for synovial sarcoma.
* There are no established causes for synovial sarcoma.
   
   
==Differentiating synovial sarcoma from other Diseases==
==Differentiating Synovial Sarcoma from Other Diseases==
*Synovial sarcoma must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
*Synovial sarcoma must be differentiated from other [[Disease|diseases]] that cause [[Arthralgia|joint pain]], mass growth, and limited [[range of motion]], such as:<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
:*[Differential dx1]
:*[[Malignant fibrous histiocytoma|Malignant fibrous histiocytoma (MFH)]]-[[fibrosarcoma]]
:*[Differential dx2]
:*[[Liposarcoma]]
:*[Differential dx3]
:*[[Osteosarcoma]]
:*[[Chondrosarcoma]]
   
   
==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of synovial sarcoma is approximately [number or range] per 100,000 individuals worldwide.
* The [[prevalence]] of synovial sarcoma remains unknown.<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
* In [year], the incidence of synovial sarcoma was estimated to be [number or range] cases per 100,000 individuals in [location].
* Synovial sarcomas account for 2.5 - 10% of all [[soft tissue]] sarcomas.
===Age===
===Age===
*Patients of all age groups may develop synovial sarcoma.
*Synovial sarcoma is more commonly observed among [[Patient|patients]] aged 15 - 40 years old.
*Synovial sarcoma is more commonly observed among [[Adolescence|adolescents]] and young [[Adult|adults]].  
*Synovial sarcoma is more commonly observed among patients aged [age range] years old.
*Synovial sarcoma is more commonly observed among [elderly patients/young patients/children].
   
   
===Gender===
===Gender===
*Synovial sarcoma affects men and women equally.
* [[Male|Males]] are more commonly affected with synovial sarcoma than [[Female|females]].
* The [[male]] to [[female]] ratio is approximately 1.2 to 1.
*[Gender 1] are more commonly affected with synovial sarcoma than [gender 2].
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
   
   
===Race===
===Race===
*There is no racial predilection for synovial sarcoma.
*There is no racial predilection for synovial sarcoma.<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
 
*Synovial sarcoma usually affects individuals of the [race 1] race.
*[Race 2] individuals are less likely to develop synovial sarcoma.
==Risk Factors==
==Risk Factors==
*Common risk factors in the development of synovial sarcoma are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
*There are no known [[Risk factor|risk factors]] associated with the development of synovial sarcoma.<ref name="pmid19865558">{{cite journal |vauthors=Gomatos IP, Alevizos L, Kafiri G, Bramis J, Leandros E |title=Management of a small incidentally discovered retroperitoneal synovial sarcoma |journal=Can J Surg |volume=52 |issue=5 |pages=E199–200 |year=2009 |pmid=19865558 |pmc=2769101 |doi= |url=}}</ref>
 
== Natural History, Complications and Prognosis==
== Screening ==
*The majority of patients with synovial sarcoma remain asymptomatic for [duration/years].  
* There is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for synovial sarcoma.
*Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
 
*If left untreated, [#%] of patients with synovial sarcoma may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
== Natural History, Complications, and Prognosis==
*Common complications of synovial sarcoma include [complication 1], [complication 2], and [complication 3].
*The majority of [[Patient|patients]] with synovial sarcoma remain [[asymptomatic]] for years.  
*Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with synovial sarcoma is approximately [#%].
*Early [[clinical]] feature includes a soft [[Palpation|palpable]] mass.  
*If left untreated, [[Patient|patients]] with synovial sarcoma may progress to develop [[Metastasis|metastases]].
*The most common [[Complication (medicine)|complication]] of synovial sarcoma is [[Lung|pulmonary]] cannonball [[Metastasis|metastases]].  
*[[Prognosis]] is generally poor, and the [[median]] survival rate of [[Patient|patients]] with synovial sarcoma is approximately 35% to 60%.
*The table below demonstrates the good and poor [[Prognosis|prognostic]] factors for [[Patient|patients]] with synovial sarcoma.<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
:{| class="wikitable"
! style="font-weight: bold;" | Poor prognosis
! style="font-weight: bold;" | Good prognosis
|-
|
*Large size (> 5 cm): most important factor
*Located in the [[trunk]] or [[head]] and [[neck]]
*Older [[Patient|patients]]
*[[Cyst|Cystic]]/[[hemorrhagic]] components
*Marked [[Heterogeneous|heterogeneity]]
*[[Histology]]
:*Poorly differentiated [[histology]]
:*Extensive [[tumor]] [[necrosis]]
:*High [[Cell nucleus|nuclear]] grade
:*[[p53]] [[Mutation|mutations]]
:*High [[Mitosis|mitotic]] rate (> 10 [[Mitosis|mitoses]] per 10 high-power field)
|
*Small size
*Located in extremity
*Younger age < 20 years of age
*Solid [[Homogeneity|homogenous]] mass
*Presence of [[calcification]]
*Biphasic [[histology]] (controversial)
|}
 
== Diagnosis ==
== Diagnosis ==
===Diagnostic Criteria===
===Diagnostic Study of Choice===
*The diagnosis of synovial sarcoma is made when at least [number] of the following [number] diagnostic criteria are met:
* The [[diagnosis]] of synovial sarcoma is typically made based on [[histology]] and is confirmed by the presence of t(X;18).<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
:*[criterion 1]
 
:*[criterion 2]
=== History and Symptoms ===
:*[criterion 3]
*Synovial sarcoma is usually [[asymptomatic]].
:*[criterion 4]
*[[Symptom|Symptoms]] of synovial sarcoma may include the following:<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
:*[[Soft tissue]] mass
=== Symptoms ===
:*Localized [[pain]]
*Synovial sarcoma is usually asymptomatic.
:*Limited [[range of motion]]
*Symptoms of synovial sarcoma may include the following:
*Specific areas of focus when obtaining the history include:
:*[symptom 1]
:*Localized [[pain]]  
:*[symptom 2]
:*Accompanying local swelling or mass, progressive [[pain]] that is not relieved with rest, night [[pain]]
:*[symptom 3]
:*Recent [[weight loss]] (or [[failure to thrive]])
:*[symptom 4]
:*Personal history of [[cancer]]
:*[symptom 5]
:*[[Family history]] of [[Bone tumor|bone tumors]]
:*[symptom 6]
 
=== Physical Examination ===
=== Physical Examination ===
*Patients with synovial sarcoma usually appear [general appearance].
*[[Patient|Patients]] with synovial sarcoma usually are well-appearing.
*Physical examination may be remarkable for:
*[[Physical examination]] may be remarkable for:
:*[finding 1]
:*[[Tenderness]] to [[palpation]]
:*[finding 2]
:*[[Soft tissue]] [[swelling]]
:*[finding 3]
:*Decreased [[range of motion]]
:*[finding 4]
:*[[Muscle atrophy]]
:*[finding 5]
:*[[Joint]] effusion
:*[finding 6]
   
   
=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with synovial sarcoma.
*There are no specific [[Medical laboratory|laboratory]] findings associated with synovial sarcoma.
 
=== Electrocardiogram ===
* There are no [[The electrocardiogram|ECG]] findings associated with synovial sarcoma.
 
=== X-ray ===
* Plain [[X-rays|x-ray]] can be normal unless the [[tumor]] is large in size or has [[Dystrophic calcification|dystrophic calcifications]].<ref>{{Cite journal
| author = [[Mark D. Murphey]], [[Michael S. Gibson]], [[Bryan T. Jennings]], [[Ana M. Crespo-Rodriguez]], [[Julie Fanburg-Smith]] & [[Donald A. Gajewski]]
| title = From the archives of the AFIP: Imaging of synovial sarcoma with radiologic-pathologic correlation
| journal = [[Radiographics : a review publication of the Radiological Society of North America, Inc]]
| volume = 26
| issue = 5
| pages = 1543–1565
| year = 2006
| month = September-October
| doi = 10.1148/rg.265065084
| pmid = 16973781
}}</ref>
 
=== Echocardiography or Ultrasound ===
* There are no [[echocardiography]] findings associated with synovial sarcoma.
* On [[ultrasound]], characteristic findings of synovial sarcoma include:<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
:*Non-specific
:*[[Heterogeneous]]
:*Hypoechoic mass
 
=== CT scan ===
*On [[Computed tomography|CT scan]], characteristic findings of synovial sarcoma include:
:*Non-specific
:*[[Soft tissue]] mass
:*[[Heterogeneous]] [[density]] and enhancement
:*[[Calcification|Calcifications]]
 
===MRI===
*[[Magnetic resonance imaging|MRI]] is the [[imaging]] modality of choice for synovial sarcoma.
*On [[Magnetic resonance imaging|MRI]], characteristic findings of synovial sarcoma include:<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
:*'''T1:''' iso- (slightly hyper-) intense to [[muscle]]/[[heterogeneous]]
:*'''T2:''' mostly hyperintense, markedly [[heterogeneous]] appearance of synovial sarcomas on [[fluid]] sensitive sequences result in so called "triple sign" which is due to areas of [[necrosis]] and [[Cyst|cystic]] degeneration with very high signal, relatively high signal [[soft tissue]] components, and areas of low signal intensity due to [[Dystrophic calcification|dystrophic calcifications]] and fibrotic bands, due to high tendency of [[Lesion|lesions]] to [[Bleeding|bleed]].
:*'''T1 C + (Gd):''' ​enhancement is usually prominent and can be diffuse (40%) [[heterogeneous]] (40%) or peripheral (20%)
*The image below demonstrates an [[Magnetic resonance imaging|MRI]] image of synovial sarcoma.
 
==Other Imaging Findings==
There are no other [[imaging]] findings associated with synovial sarcoma.
 
==Other Diagnostic studies==
There are no other [[Diagnosis|diagnostic]] studies associated with synovial sarcoma.


*A  [positive/negative] [test name] is diagnostic of synovial sarcoma.
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of synovial sarcoma.
*Other laboratory findings consistent with the diagnosis of synovial sarcoma include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
===Imaging Findings===
*There are no [imaging study] findings associated with synovial sarcoma.
*[Imaging study 1] is the imaging modality of choice for synovial sarcoma.
*On [imaging study 1], synovial sarcoma is characterized by [finding 1], [finding 2], and [finding 3].
*[Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].
=== Other Diagnostic Studies ===
*Synovial sarcoma may also be diagnosed using [diagnostic study name].
*Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].
== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*There is no treatment for synovial sarcoma; the mainstay of therapy is supportive care.
*The mainstay of [[therapy]] for synovial sarcoma includes:<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
:*[[Doxorubicin]]
*The mainstay of therapy for synovial sarcoma is [medical therapy 1] and [medical therapy 2].
:*[[Ifosfamide]]
*[Medical therapy 1] acts by [mechanism of action1].
:*[[Gemcitabine]]
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
 
=== Surgery ===
=== Surgery ===
*Surgery is the mainstay of therapy for synovial sarcoma.
*[[Surgery]] is the mainstay of [[therapy]] for synovial sarcoma.<ref name="pmid19865558">{{cite journal |vauthors=Gomatos IP, Alevizos L, Kafiri G, Bramis J, Leandros E |title=Management of a small incidentally discovered retroperitoneal synovial sarcoma |journal=Can J Surg |volume=52 |issue=5 |pages=E199–200 |year=2009 |pmid=19865558 |pmc=2769101 |doi= |url=}}</ref>
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of synovial sarcoma.
*[[Surgery|Surgical]] [[resection]] in conjunction with [[chemotherapy]] or [[Radiation therapy|radiation]] is the most common approach to the treatment of synovial sarcoma.<ref name="pmid9930576">{{cite journal |vauthors=Fisher C |title=Synovial sarcoma |journal=Ann Diagn Pathol |volume=2 |issue=6 |pages=401–21 |year=1998 |pmid=9930576 |doi= |url=}}</ref>
*[Surgical procedure] can only be performed for patients with [disease stage] synovial sarcoma.
   
   
=== Prevention ===
=== Primary Prevention ===
*There are no primary preventive measures available for synovial sarcoma.
*There are no established measures for the [[Prevention (medical)|primary prevention]] of synovial sarcoma.
 
*Effective measures for the primary prevention of synovial sarcoma include [measure1], [measure2], and [measure3].
===Secondary Prevention===
There are no established measures for the [[Prevention (medical)|secondary prevention]] of synovial sarcoma.


*Once diagnosed and successfully treated, patients with synovial sarcoma are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
   
   
[[Category: Oncology]]
[[Category: Oncology]]
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Orthopedics]]

Latest revision as of 18:40, 1 June 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: Malignant synovioma

Overview

Synovial sarcoma (also known as malignant synovioma) is a very rare form of soft tissue sarcoma, which usually occurs near the joints in upper and lower extremities. Synovial sarcoma was first discovered by Pack and Tabah in 1955. Synovial sarcoma may be classified according to histopathological findings into 4 sub-types: biphasic, monophasic fibrous (most common), monophasic epithelial, and poorly differentiated. The pathogenesis of synovial sarcoma is characterized by the dysregulation of gene expression of SYT-SSX gene. The most common locations for the occurrence of synovial sarcoma include Knee, adjacent to large joints, and Popliteal fossa. The SYT-SSX fusion gene (located in chromosome 18) has been associated with the development of synovial sarcoma. There are no established causes for synovial sarcoma. Synovial sarcoma must be differentiated from other diseases that cause joint pain, mass growth, and limited range of motion, such as malignant fibrous histiocytoma (MFH)-fibrosarcoma, Liposarcoma, Osteosarcoma, and Chondrosarcoma. The prevalence of synovial sarcoma remains unknown. Synovial sarcomas account for 2.5 - 10% of all soft tissue sarcomas. Synovial sarcoma is more commonly observed among patients aged 15 - 40 years old. There is no racial predilection for synovial sarcoma. There are no known risk factors associated with the development of synovial sarcoma. There is insufficient evidence to recommend routine screening for synovial sarcoma. The majority of patients with synovial sarcoma remain asymptomatic for years. If left untreated, patients with synovial sarcoma may progress to develop metastases. Prognosis is generally poor, and the median survival rate of patients with synovial sarcoma is approximately 35% to 60%. The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18). There are no specific laboratory findings associated with synovial sarcoma. Patients with synovial sarcoma usually are well-appearing. There are no specific laboratory findings associated with synovial sarcoma. There are no ECG findings associated with synovial sarcoma. Plain x-ray can be normal unless the tumor is large in size or has dystrophic calcifications. On ultrasound, characteristic findings of synovial sarcoma include heterogeneity and hypo-echoic mass. On CT scan, characteristic findings of synovial sarcoma include Soft tissue mass, calcifications, and Heterogeneous density and enhancement. MRI is the imaging modality of choice for synovial sarcoma. Medical therapy include Doxorubicin, ifosfamide, and gemcitabine. Surgery is the mainstay of therapy. Surgical resection in conjunction with chemotherapy or radiation is the most common approach to the treatment of synovial sarcoma. There are no established measures for the prevention of synovial sarcoma.

Historical Perspective

  • Synovial sarcoma was first discovered by Pack and Tabah in 1955.[1]

Classification

  • Biphasic
  • Monophasic fibrous (most common)
  • Monophasic epithelial
  • Poorly differentiated

Pathophysiology

  • The pathogenesis of synovial sarcoma is characterized by the dysregulation of gene expression of SYT-SSX gene.[2]
  • The most common locations for the occurrence of synovial sarcoma include:[1]
  • The SYT-SSX fusion gene (located in chromosome 18) has been associated with the development of synovial sarcoma.
  • On gross pathology, characteristic findings of synovial sarcoma include:
  • Solid often lobulated
  • Grey-yellow
  • Pushing border to ill-defined border

Causes

  • There are no established causes for synovial sarcoma.

Differentiating Synovial Sarcoma from Other Diseases

Epidemiology and Demographics

  • The prevalence of synovial sarcoma remains unknown.[2]
  • Synovial sarcomas account for 2.5 - 10% of all soft tissue sarcomas.

Age

  • Synovial sarcoma is more commonly observed among patients aged 15 - 40 years old.
  • Synovial sarcoma is more commonly observed among adolescents and young adults.

Gender

  • Males are more commonly affected with synovial sarcoma than females.
  • The male to female ratio is approximately 1.2 to 1.

Race

  • There is no racial predilection for synovial sarcoma.[2]

Risk Factors

  • There are no known risk factors associated with the development of synovial sarcoma.[1]

Screening

  • There is insufficient evidence to recommend routine screening for synovial sarcoma.

Natural History, Complications, and Prognosis

Poor prognosis Good prognosis

Diagnosis

Diagnostic Study of Choice

  • The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18).[2]

History and Symptoms

  • Specific areas of focus when obtaining the history include:

Physical Examination

Laboratory Findings

  • There are no specific laboratory findings associated with synovial sarcoma.

Electrocardiogram

  • There are no ECG findings associated with synovial sarcoma.

X-ray

Echocardiography or Ultrasound

CT scan

  • On CT scan, characteristic findings of synovial sarcoma include:

MRI

  • MRI is the imaging modality of choice for synovial sarcoma.
  • On MRI, characteristic findings of synovial sarcoma include:[2]
  • T1: iso- (slightly hyper-) intense to muscle/heterogeneous
  • T2: mostly hyperintense, markedly heterogeneous appearance of synovial sarcomas on fluid sensitive sequences result in so called "triple sign" which is due to areas of necrosis and cystic degeneration with very high signal, relatively high signal soft tissue components, and areas of low signal intensity due to dystrophic calcifications and fibrotic bands, due to high tendency of lesions to bleed.
  • T1 C + (Gd): ​enhancement is usually prominent and can be diffuse (40%) heterogeneous (40%) or peripheral (20%)
  • The image below demonstrates an MRI image of synovial sarcoma.

Other Imaging Findings

There are no other imaging findings associated with synovial sarcoma.

Other Diagnostic studies

There are no other diagnostic studies associated with synovial sarcoma.

Treatment

Medical Therapy

  • The mainstay of therapy for synovial sarcoma includes:[2]

Surgery

Primary Prevention

Secondary Prevention

There are no established measures for the secondary prevention of synovial sarcoma.

References

  1. ↑ 1.0 1.1 1.2 1.3 Gomatos IP, Alevizos L, Kafiri G, Bramis J, Leandros E (2009). "Management of a small incidentally discovered retroperitoneal synovial sarcoma". Can J Surg. 52 (5): E199–200. PMC 2769101. PMID 19865558.
  2. ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 2.12 Fisher C (1998). "Synovial sarcoma". Ann Diagn Pathol. 2 (6): 401–21. PMID 9930576.
  3. ↑ Mark D. Murphey, Michael S. Gibson, Bryan T. Jennings, Ana M. Crespo-Rodriguez, Julie Fanburg-Smith & Donald A. Gajewski (2006). "From the archives of the AFIP: Imaging of synovial sarcoma with radiologic-pathologic correlation". Radiographics : a review publication of the Radiological Society of North America, Inc. 26 (5): 1543–1565. doi:10.1148/rg.265065084. PMID 16973781. Unknown parameter |month= ignored (help)
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