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{{DiseaseDisorder infobox |
__NOTOC__
  Name        = Synovial sarcoma |
{{SI}}                                                                     
  ICD10      = |
{{CMG}} {{AE}} {{MV}}
  ICD9        = |
  ICDO        = 9040/3-9043/3 |
{{SK}} Malignant synovioma
}}
{{SI}}
==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==
*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>


==Introduction==
==Classification==
'''Synovial sarcoma''' is a rare soft-tissue [[sarcoma]] defined by the presence of a unique translocation, t(X;18)(q11.2;p11.2). It usually occurs adjacent to [[joint]]s in the [[limb]]s and was originally thought to arise from [[synovium]].
*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
:*Monophasic [[fibrous]] (most common)
:*Monophasic [[Epithelium|epithelial]]
:*Poorly differentiated


However, synovial sarcoma is a misnomer and the origin of the tissue is unknown. Primary synovial sarcomas have been documented in most human tissues and organs, including the brain, prostate and heart. Synovial sarcoma occurs most commonly in adolescents and young adults. Diagnosis is based on [[histology]]; there are three major histologic subtypes: monophasic (consisting on sheets of spindle cells), biphasic (spindle cells admixed with areas of epithelial differentiation) and poorly differentiated. Immunohistochemical techniques can help, however the gold standard for diagnosing synovial sarcoma is detection of t(X;18) by cytogenetics, FISH or RT-PCR. Despite treatment, prognosis is generally poor, with approximately 50% of patients dying from local reccurrence or [[metastasis]] within 10 years.
==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 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]]
:*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==
* There are no established causes for synovial sarcoma.
==Differentiating Synovial Sarcoma from Other Diseases==
*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|Malignant fibrous histiocytoma (MFH)]]-[[fibrosarcoma]]
:*[[Liposarcoma]]
:*[[Osteosarcoma]]
:*[[Chondrosarcoma]]
==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>
* Synovial sarcomas account for 2.5 - 10% of all [[soft tissue]] sarcomas.
===Age===
*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]].  
===Gender===
* [[Male|Males]] are more commonly affected with synovial sarcoma than [[Female|females]].
* The [[male]] to [[female]] ratio is approximately 1.2 to 1.
===Race===
*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==
*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>
 
== Screening ==
* There is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for synovial sarcoma.


==Causes==
== Natural History, Complications, and Prognosis==
Synovial sarcoma is defined by the presence of the SYT-SSX fusion gene, the result of a translocation event between the SYT gene on chromosome 18 and one of 3 SSX genes (SSX1, SSX2 and SSX4) on chromosome X. The diagnosis of synovial sarcoma is typically made based on histology and is confirmed by the presence of t(X;18). The SYT-SSX fusion protein that results from this translocation brings together the transcriptional activating domain of SYT and the transcriptional repressor domains of SSX. SYT-SSX is thought to underlie synovial sarcoma pathogenesis through dysregulation of gene expression. Gene expression studies have identified a pattern of gene expression typical of synovial sarcoma. The exact function(s) of the fusion protein remain unclear. Recent research has offered some insight into synovial sarcoma development. The genes identified in expression studies resemble those activated in developemnt of the neural crest, an area of the vertebrate embryo that eventually gives rise to various tissues, including nerves and cartilage. SYT-SSX has be shown to interact with transcriptional regulators involved in cell differentiation and proliferation. Interestingly, when inserted into non-synovial sarcoma cell lines, SYT-SSX has been shown to be tumorigenic in some but not others, suggesting that SYT-SSX oncogenesis occurs only in an approriate cellular context. Further investigation of the molecular mechanisms underlying SYT-SSX function will likely lead to not only a better understanding of this disease but improved diagnostic and treatment tools for this tumor.
*The majority of [[Patient|patients]] with synovial sarcoma remain [[asymptomatic]] for years.
*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 ==
===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>


==Symptoms==
=== History and Symptoms ===
General symptoms related to maligancies are reported (fatigue, fever, etc.) however synovial sarcoma usually presents with an otherwsie asymptomatic swelling or mass. Pain, discomfort or inhibition or loss of function in the area of the tumor may also be reported. Symptoms related to metastases are usually site-specific and related to mass effects.  The [[diagnosis]] of synovial sarcoma is made by histology, immunohistochemistry and, if necessary and possible, by demonstration of t(X;18).
*Synovial sarcoma is usually [[asymptomatic]].
*[[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
:*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 tumor|bone tumors]]


==Treatment==
=== Physical Examination ===
Treatment usually involves:
*[[Patient|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 [[Medical laboratory|laboratory]] findings associated with synovial sarcoma.


*[[Surgery]], to remove the tumor and a safety margin of healthy tissue. This is the mainstay of synovial sarcoma treatment and is curative in approximately 20-70% of patients, depending on the particular study being quoted.
=== Electrocardiogram ===
*Conventional [[chemotherapy]], (for example, [[Doxorubicin hydrochloride]] and [[Ifosfamide]]), to reduce the number of remaining microscopic cancer cells. The benefit of chemotherapy in synovial sarcoma to overall survival remains unclear, although a recent study has shown that survival of patients with advanced, poorly differentiated disease marginally improves with doxorubicin/ifosfamide treatment.
* There are no [[The electrocardiogram|ECG]] findings associated with synovial sarcoma.
*[[Radiotherapy]] to reduce the chance of local recurrence. The benefit of radiotherapy in this disease is less clear than for chemotherapy.


Recent laboratory-based studies have identified a number of potential systemic therapies that may prove more effecacious than conventional chemotherapy and improve survival, however the rarity of this tumor makes clinical trial organization difficult and validation of these experimental therapeutics will likely be long in coming.
=== 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>


==Scans to be undertaken before, during, and after treatment==
=== Echocardiography or Ultrasound ===
Various scanning techniques can be used to further localise and identify this cancer:
* 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


*[[X-ray]]
=== CT scan ===
*[[computed tomography|CT]]
*On [[Computed tomography|CT scan]], characteristic findings of synovial sarcoma include:
*[[MRI]]
:*Non-specific
:*[[Soft tissue]] mass
:*[[Heterogeneous]] [[density]] and enhancement
:*[[Calcification|Calcifications]]


During treatment, the patient may have [[Bone Density]] Scans, to measure the impact of the chemotherapy on the skeleton.
===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.


==External links==
==Other Imaging Findings==
*[http://www.webmd.com WebMD]
There are no other [[imaging]] findings associated with synovial sarcoma.
*[http://www.emedicine.com eMedicine]
*[http://www.liddyshriversarcomainitiative.org/Newsletters/V02N06/Synovial/synovial.htm Synovial Sarcoma by Andrea Ferrari, MD and Paolo Collini MD]


{{Soft tissue tumors and sarcomas}}
==Other Diagnostic studies==
There are no other [[Diagnosis|diagnostic]] studies associated with synovial sarcoma.


[[Category:Oncology]]
== Treatment ==
=== 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>
:*[[Doxorubicin]]
:*[[Ifosfamide]]
:*[[Gemcitabine]]


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


{{WikiDoc Help Menu}}
===Secondary Prevention===
There are no established measures for the [[Prevention (medical)|secondary prevention]] of synovial sarcoma.


{{WikiDoc Sources}}
==References==
{{Reflist|2}}
[[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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