Synovial sarcoma: Difference between revisions
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==Overview== | ==Overview== | ||
'''Synovial sarcoma''' (also known as '''malignant synovioma''') is a very rare form of soft tissue sarcoma, which usually occurs near to the joints in upper and lower extremities. | '''Synovial sarcoma''' (also known as '''malignant synovioma''') is a very rare form of soft tissue sarcoma, which usually occurs near to 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 subtypes: 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 location for these synovial sarcoma, include: | ||
knee, adjacent to large joints, and popliteal fossa.<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 SYT-SSX fusion gene (located in chromosome 18) has been associated with the development of synovial sarcoma. Synovial sarcomas account for 2.5-10% of all soft tissue sarcoma, and it is more commonly observed among adolescents and young adults. 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> MRI is the imaging modality of choice for synovial sarcoma. Surgical resection in conjunction with chemotherapy or 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> | |||
==Historical Perspective== | ==Historical Perspective== | ||
*Synovial sarcoma was first discovered by | *Synovial sarcoma was first discovered by Pack and Tabah in 1955. | ||
==Classification== | ==Classification== | ||
*Synovial sarcoma may be classified according to histopathological findings into 4 subtypes: | *Synovial sarcoma may be classified according to histopathological findings into 4 subtypes:<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> | ||
:*Biphasic | :*Biphasic | ||
:*Monophasic fibrous (most common) | :*Monophasic fibrous (most common) | ||
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==Pathophysiology== | ==Pathophysiology== | ||
*The pathogenesis of synovial sarcoma is characterized by the dysregulation of gene expression of SYT-SSX gene. | *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 most common location for these synovial sarcoma, include: | *The most common location for these synovial sarcoma, include: | ||
:*Knee | :*Knee | ||
:*Adjacent to large joints | :*Adjacent to large joints | ||
:*Popliteal fossa | :*Popliteal fossa | ||
*The SYT-SSX fusion gene (chromosome 18) has been associated with the development of synovial sarcoma. | *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: | *On gross pathology, characteristic findings of synovial sarcoma, include: | ||
:*Solid often lobulated | :*Solid often lobulated | ||
:*Grey-yellow | :*Grey-yellow | ||
:*Pushing border to ill-defined border | :*Pushing border to ill-defined border | ||
*On microscopic histopathological analysis, characteristic findings of synovial sarcoma, include: | *On microscopic 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 | :*Non-specific appearances | ||
:*Well or poorly defined heterogeneous masses | :*Well or poorly defined heterogeneous masses | ||
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==Differentiating Synovial Sarcoma from Other Diseases== | ==Differentiating Synovial Sarcoma from Other Diseases== | ||
*Synovial sarcoma must be differentiated from other diseases that cause joint pain, mass growth, and limited range of motion, such as: | *Synovial sarcoma must be differentiated from other diseases that cause 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> | ||
:*Malignant fibrous histiocytoma (MFH)-fibrosarcoma | :*Malignant fibrous histiocytoma (MFH)-fibrosarcoma | ||
:*Liposarcoma | :*Liposarcoma | ||
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==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
* The prevalence of synovial sarcoma remains unknown. | * 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> | ||
* Synovial sarcomas account for 2.5-10% of all soft tissue sarcoma | * Synovial sarcomas account for 2.5-10% of all soft tissue sarcoma | ||
===Age=== | ===Age=== | ||
| Line 58: | Line 59: | ||
===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> | ||
==Risk Factors== | ==Risk Factors== | ||
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*The most common complications of synovial sarcoma is pulmonary cannonball metastases. | *The most common complications of synovial sarcoma is pulmonary cannonball metastases. | ||
*Prognosis is generally poor, and the median survival rate of patients with synovial sarcoma is approximately 35% to 60%. | *Prognosis is generally poor, and the median survival rate of patients with synovial sarcoma is approximately 35% to 60%. | ||
*The table below demonstrates the good and poor prognostic factors for patients with synovial sarcoma. | *The table below demonstrates the good and poor prognostic factors for 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" | :{| class="wikitable" | ||
! style="font-weight: bold;" | Poor prognosis | ! style="font-weight: bold;" | Poor prognosis | ||
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== Diagnosis == | == Diagnosis == | ||
===Diagnostic Criteria=== | ===Diagnostic Criteria=== | ||
* The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18). | * 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> | ||
=== Symptoms === | === Symptoms === | ||
*Synovial sarcoma is usually asymptomatic. | *Synovial sarcoma is usually asymptomatic. | ||
*Symptoms of synovial sarcoma may include the following: | *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 | :*Soft tissue mass | ||
:*Localized pain | :*Localized pain | ||
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===Imaging Findings=== | ===Imaging Findings=== | ||
*MRI is the imaging modality of choice for synovial sarcoma. | *MRI is the imaging modality of choice for synovial sarcoma. | ||
*On ultrasound, characteristic findings of synovial sarcoma, include: | *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 | :*Non-specific | ||
:*Heterogeneous | :*Heterogeneous | ||
| Line 135: | Line 136: | ||
:*Heterogeneous density and enhancement | :*Heterogeneous density and enhancement | ||
:*Calcifications | :*Calcifications | ||
*On MRI, characteristic findings of synovial sarcoma, include: | *On 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 | :*T1: iso- (slightly hyper-) intense to muscle/ heterogeneous | ||
:*T2: mostly hyperintense, markedly heterogeneous appearance of synovial cell 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, there might be areas of fluid-fluid levels known as "bowl of grapes" are seen in up to 10-25% of cases | :*T2: mostly hyperintense, markedly heterogeneous appearance of synovial cell 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, there might be areas of fluid-fluid levels known as "bowl of grapes" are seen in up to 10-25% of cases | ||
| Line 142: | Line 143: | ||
== Treatment == | == Treatment == | ||
=== Medical Therapy === | === Medical Therapy === | ||
*The mainstay of therapy for synovial sarcoma, includes: | *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 | :*Doxorubicin | ||
:*Ifosfamide | :*Ifosfamide | ||
| Line 149: | Line 150: | ||
=== Surgery === | === Surgery === | ||
*Surgery is the mainstay of therapy for synovial sarcoma. | *Surgery is the mainstay of therapy for synovial sarcoma. | ||
*Surgical resection in conjunction with chemotherapy or radiation is the most common approach to the treatment of synovial sarcoma. | *Surgical resection in conjunction with chemotherapy or 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> | ||
=== Prevention === | === Prevention === | ||
Revision as of 21:16, 19 May 2016
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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 to 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 subtypes: 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 location for these synovial sarcoma, include: knee, adjacent to large joints, and popliteal fossa.[1] The SYT-SSX fusion gene (located in chromosome 18) has been associated with the development of synovial sarcoma. Synovial sarcomas account for 2.5-10% of all soft tissue sarcoma, and it is more commonly observed among adolescents and young adults. The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18).[1] MRI is the imaging modality of choice for synovial sarcoma. Surgical resection in conjunction with chemotherapy or radiation is the most common approach to the treatment of synovial sarcoma.[1]
Historical Perspective
- Synovial sarcoma was first discovered by Pack and Tabah in 1955.
Classification
- Synovial sarcoma may be classified according to histopathological findings into 4 subtypes:[1]
- 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.[1]
- The most common location for these synovial sarcoma, include:
- Knee
- Adjacent to large 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 lobulated
- Grey-yellow
- Pushing border to ill-defined border
- On microscopic histopathological analysis, characteristic findings of synovial sarcoma, include:[1]
- Non-specific appearances
- Well or poorly defined heterogeneous masses
- Frequent areas of hemorrhage
- Necrosis
Causes
- There are no established causes for synovial sarcoma.
Differentiating Synovial Sarcoma from Other Diseases
- Synovial sarcoma must be differentiated from other diseases that cause joint pain, mass growth, and limited range of motion, such as:[1]
- Malignant fibrous histiocytoma (MFH)-fibrosarcoma
- Liposarcoma
- Osteosarcoma
- Chondrosarcoma
Epidemiology and Demographics
- The prevalence of synovial sarcoma remains unknown.[1]
- Synovial sarcomas account for 2.5-10% of all soft tissue sarcoma
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.[1]
Risk Factors
- There are no known risk factors associated with the development of synovial sarcoma.
Natural History, Complications and Prognosis
- The majority of patients with synovial sarcoma remain asymptomatic for years.
- Early clinical feature includes a soft palpable mass.
- If left untreated, patients with synovial sarcoma may progress to develop metastases
- The most common complications of synovial sarcoma is pulmonary cannonball metastases.
- Prognosis is generally poor, and the median survival rate of patients with synovial sarcoma is approximately 35% to 60%.
- The table below demonstrates the good and poor prognostic factors for patients with synovial sarcoma.[1]
Poor prognosis Good prognosis - Large size (>5 cm): most important factor
- Located in the trunk or head and neck
- Older patients
- Cystic/haemorrhagic components
- Marked heterogeneity
- Histology
- Poorly differentiated histology
- Extensive tumour necrosis
- High nuclear grade
- p53 mutations
- High mitotic rate (>10 mitoses per 10 high-power field)
- Small size
- Located in extremity
- Younger age <20 years of age
- Solid homogenous mass
- Presence of calcification
- Biphasic histology (controversial)
Diagnosis
Diagnostic Criteria
- The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18).[1]
Symptoms
- Synovial sarcoma is usually asymptomatic.
- Symptoms of synovial sarcoma may include the following:[1]
- Soft tissue mass
- Localized pain
- Limited range of motion
- Specific areas of focus when obtaining the history, include:
- Localized pain
- Accompanying local swelling or mass, progressive pain that is not relieved with rest, night pain
- Recent weight loss (or failure to thrive)
- Personal history of cancer
- Family history of bone tumors
Physical Examination
- Patients with synovial sarcoma usually are well-appearing.
- Physical examination may be remarkable for:
- Tenderness to palpation
- Soft tissue swelling
- Decreased range of motion
- Muscle atrophy
- Joint effusion
Laboratory Findings
- There are no specific laboratory findings associated with synovial sarcoma.
Imaging Findings
- MRI is the imaging modality of choice for synovial sarcoma.
- On ultrasound, characteristic findings of synovial sarcoma, include:[1]
- Non-specific
- Heterogeneous
- Hypoechoic mass
- On CT, characteristic findings of synovial sarcoma, include:
- Non-specific
- Soft tissue mass
- Heterogeneous density and enhancement
- Calcifications
- On MRI, characteristic findings of synovial sarcoma, include:[1]
- T1: iso- (slightly hyper-) intense to muscle/ heterogeneous
- T2: mostly hyperintense, markedly heterogeneous appearance of synovial cell 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, there might be areas of fluid-fluid levels known as "bowl of grapes" are seen in up to 10-25% of cases
- T1 C + (Gd): enhancement is usually prominent and can be diffuse (40%) heterogeneous (40%) or peripheral (20%)
Treatment
Medical Therapy
- The mainstay of therapy for synovial sarcoma, includes:[1]
- Doxorubicin
- Ifosfamide
- Gemcitabine
Surgery
- Surgery is the mainstay of therapy for synovial sarcoma.
- Surgical resection in conjunction with chemotherapy or radiation is the most common approach to the treatment of synovial sarcoma.[1]
Prevention
- There are no primary preventive measures available for synovial sarcoma.