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 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:
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.
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 Pack and Tabah in 1955.  
*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>


==Classification==
==Classification==
*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>
*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>
:*Biphasic
:*Biphasic
:*Monophasic fibrous (most common)
:*Monophasic [[fibrous]] (most common)
:*Monophasic epithelial
:*Monophasic [[Epithelium|epithelial]]
:*Poorly differentiated
:*Poorly differentiated


==Pathophysiology==
==Pathophysiology==
*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 [[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 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>
:*Knee
:*[[Knee]]
:*Adjacent to large joints
:*Adjacent to large [[Joint|joints]]
:*Popliteal fossa
:*[[Popliteal fossa]]
*The SYT-SSX fusion gene (located in 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 [[Lobule|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:<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>
*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
:*Non-specific appearances
:*Well or poorly defined heterogeneous masses
:*Well or poorly defined [[heterogeneous]] masses
:*Frequent areas of hemorrhage
:*Frequent areas of [[Bleeding|hemorrhage]]
:*Necrosis  
:*[[Necrosis]]
   
   
==Causes==
==Causes==
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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:<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 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>
:*Malignant fibrous histiocytoma (MFH)-fibrosarcoma
:*[[Malignant fibrous histiocytoma|Malignant fibrous histiocytoma (MFH)]]-[[fibrosarcoma]]
:*Liposarcoma
:*[[Liposarcoma]]
:*Osteosarcoma
:*[[Osteosarcoma]]
:*Chondrosarcoma
:*[[Chondrosarcoma]]
   
   
==Epidemiology and Demographics==
==Epidemiology and Demographics==
* 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>
* 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]] sarcomas.
===Age===
===Age===
*Synovial sarcoma is more commonly observed among patients aged 15-40 years old.
*Synovial sarcoma is more commonly observed among [[Patient|patients]] aged 15 - 40 years old.
*Synovial sarcoma is more commonly observed among adolescents and young adults.  
*Synovial sarcoma is more commonly observed among [[Adolescence|adolescents]] and young [[Adult|adults]].  
   
   
===Gender===
===Gender===
* Males are more commonly affected with synovial sarcoma than females
* [[Male|Males]] are more commonly affected with synovial sarcoma than [[Female|females]].
* The male to female ratio is approximately 1.2 to 1.
* The [[male]] to [[female]] ratio is approximately 1.2 to 1.
   
   
===Race===
===Race===
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==Risk Factors==
==Risk Factors==
*There are no known risk factors associated with the development of synovial sarcoma.
*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 years.  
* There is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for synovial sarcoma.
*Early clinical feature includes a soft palpable mass.  
 
*If left untreated, patients with synovial sarcoma may progress to develop metastases
== Natural History, Complications, and Prognosis==
*The most common complications of synovial sarcoma is pulmonary cannonball metastases.  
*The majority of [[Patient|patients]] with synovial sarcoma remain [[asymptomatic]] for years.  
*Prognosis is generally poor, and the median survival rate of patients with synovial sarcoma is approximately 35% to 60%.
*Early [[clinical]] feature includes a soft [[Palpation|palpable]] mass.  
*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>
*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"
:{| class="wikitable"
! style="font-weight: bold;" | Poor prognosis
! style="font-weight: bold;" | Poor prognosis
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|-
|-
|  
|  
*Large size (>5 cm): most important factor
*Large size (> 5 cm): most important factor
*Located in the trunk or head and neck
*Located in the [[trunk]] or [[head]] and [[neck]]
*Older patients
*Older [[Patient|patients]]
*Cystic/haemorrhagic components
*[[Cyst|Cystic]]/[[hemorrhagic]] components
*Marked heterogeneity
*Marked [[Heterogeneous|heterogeneity]]
*Histology  
*[[Histology]]
:*Poorly differentiated histology
:*Poorly differentiated [[histology]]
:*Extensive tumour necrosis
:*Extensive [[tumor]] [[necrosis]]
:*High nuclear grade
:*High [[Cell nucleus|nuclear]] grade
:*p53 mutations
:*[[p53]] [[Mutation|mutations]]
:*High mitotic rate (>10 mitoses per 10 high-power field)
:*High [[Mitosis|mitotic]] rate (> 10 [[Mitosis|mitoses]] per 10 high-power field)
|  
|  
*Small size
*Small size
*Located in extremity
*Located in extremity
*Younger age <20 years of age
*Younger age < 20 years of age
*Solid homogenous mass
*Solid [[Homogeneity|homogenous]] mass
*Presence of calcification
*Presence of [[calcification]]
*Biphasic histology (controversial)
*Biphasic [[histology]] (controversial)
|}
|}


== Diagnosis ==
== Diagnosis ==
===Diagnostic Criteria===
===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).<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 [[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 ===
=== History and Symptoms ===
*Synovial sarcoma is usually asymptomatic.
*Synovial sarcoma is usually [[asymptomatic]].
*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>
*[[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
:*[[Soft tissue]] mass
:*Localized pain
:*Localized [[pain]]
:*Limited range of motion
:*Limited [[range of motion]]
*Specific areas of focus when obtaining the history, include:
*Specific areas of focus when obtaining the history include:
:*Localized pain  
:*Localized [[pain]]
:*Accompanying local swelling or mass, progressive pain that is not relieved with rest, night pain
:*Accompanying local swelling or mass, progressive [[pain]] that is not relieved with rest, night [[pain]]
:*Recent weight loss (or failure to thrive)
:*Recent [[weight loss]] (or [[failure to thrive]])
:*Personal history of cancer
:*Personal history of [[cancer]]
:*Family history of bone tumors
:*[[Family history]] of [[Bone tumor|bone tumors]]


=== Physical Examination ===
=== Physical Examination ===
*Patients with synovial sarcoma usually are well-appearing.
*[[Patient|Patients]] with synovial sarcoma usually are well-appearing.
*Physical examination may be remarkable for:
*[[Physical examination]] may be remarkable for:
:*Tenderness to palpation
:*[[Tenderness]] to [[palpation]]
:*Soft tissue swelling
:*[[Soft tissue]] [[swelling]]
:*Decreased range of motion
:*Decreased [[range of motion]]
:*Muscle atrophy
:*[[Muscle atrophy]]
:*Joint effusion
:*[[Joint]] effusion
   
   
=== 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.
 
===Imaging Findings===
=== Electrocardiogram ===
*MRI is the imaging modality of choice for synovial sarcoma.
* There are no [[The electrocardiogram|ECG]] 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>
 
=== 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  
:*Non-specific  
:*Heterogeneous  
:*[[Heterogeneous]]
:*Hypoechoic mass
:*Hypoechoic mass
*On CT, characteristic findings of synovial sarcoma, include:  
 
=== CT scan ===
*On [[Computed tomography|CT scan]], characteristic findings of synovial sarcoma include:  
:*Non-specific
:*Non-specific
:*Soft tissue mass  
:*[[Soft tissue]] mass  
:*Heterogeneous density and enhancement
:*[[Heterogeneous]] [[density]] and enhancement
:*Calcifications
:*[[Calcification|Calcifications]]
*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
===MRI===
:*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
*[[Magnetic resonance imaging|MRI]] is the [[imaging]] modality of choice for synovial sarcoma.
:*T1 C + (Gd): ​enhancement is usually prominent and can be diffuse (40%) heterogeneous (40%) or peripheral (20%)
*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>
*The image below demonstrates an MRI image of synovial sarcoma.  
:*'''T1:''' iso- (slightly hyper-) intense to [[muscle]]/[[heterogeneous]]
<gallery>
:*'''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]].
Image:Synovial-sarcoma-2.jpg| MRI:Synovial sarcoma
:*'''T1 C + (Gd):''' ​enhancement is usually prominent and can be diffuse (40%) [[heterogeneous]] (40%) or peripheral (20%)
</gallery>
*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.


== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*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>
*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]]
:*Gemcitabine
:*[[Gemcitabine]]


=== 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 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>
*[[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>
   
   
=== 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.
 
===Secondary Prevention===
There are no established measures for the [[Prevention (medical)|secondary prevention]] of synovial sarcoma.


==References==
==References==
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[[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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