Synovial sarcoma

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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.[1] 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.[2] 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).[2] 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.[2]

Historical Perspective

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

Classification

  • Synovial sarcoma may be classified according to histopathological findings into 4 subtypes:[2]
  • 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 location for these 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
  • On microscopic histopathological analysis, characteristic findings of synovial sarcoma, include:[2]
  • 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:[2]

Epidemiology and Demographics

  • The prevalence of synovial sarcoma remains unknown.[2]
  • 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.[2]

Risk Factors

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

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.[2]
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).[2]

Symptoms

  • Synovial sarcoma is usually asymptomatic.
  • Symptoms of synovial sarcoma may include the following:[2]
  • 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:[2]
  • 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:[2]
  • 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%)
  • The image below demonstrates an MRI image of synovial sarcoma.

Treatment

Medical Therapy

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

Surgery

  • Surgery is the mainstay of therapy for synovial sarcoma.[1]
  • Surgical resection in conjunction with chemotherapy or radiation is the most common approach to the treatment of synovial sarcoma.[2]

Prevention

  • There are no primary preventive measures available for synovial sarcoma.

References

  1. 1.0 1.1 1.2 1.3 1.4 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 2769101Freely accessible. 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 2.13 2.14 2.15 Fisher C (1998). "Synovial sarcoma". Ann Diagn Pathol. 2 (6): 401–21. PMID 9930576. 

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