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Synovial Sarcoma

Topic updated on 06/11/14 10:13am
  
Introduction
  • Malignant soft tissue sarcoma which arises near joints, but rarely within the joint
    • cellular origin of synovial sarcoma is unknown, but it is not the synovial cell or any cell involved in the synovium
      • the name synovial sarcoma is a misnomer.
  • Epidemiology  
    • demographics
      • most common sarcoma found in young adults (15-40 years) 
      • affects more males than females
    • location
      • it is the most common malignant sarcoma of the foot 
  • Genetics
    • chromosomal translocation t(X;18) is observed in more than 90% of cases 
    • translocation forms the SYT-SSX1, 2, or 4 fusion protein 
  • Metastatsis
    • synovial sarcoma typically shows high histologic grade
    • metastasis may develop in 30-60% of patients
      • like other sarcomas, the lung is most common site of metastasis
      • synovial sarcoma is one of the rare soft tissue sarcomas which can metastasize to lymph nodes 
        • the other sarcomas which can metastatize to lymph nodes include, epitheliod sarcoma, angiosarcoma, rhabdomyosarcoma, clear cell sarcoma
        • can stage with lymph node biopsy
          • while lymph node metastasis is a poor prognostic sign, it is not as bad as lung metastasis
    • metastasis is more common with large, deep, and high grade sarcomas
  • Prognosis
    • overall prognosis is poor
      • 5 year survival is approximately 50%
      • 10 year survival is approximately 25%
Presentation
  • Symptoms
    • typically present as a growing mass in proximity to a joint
    • may be painless or painful
    • most commonly occur in para-articular locations
      • knee, shoulder, elbow, foot
      • 60% are found in the lower extremity
  • Physical exam
    • examen for regional lymphadenopathy
Imaging
  • Radiographs
    • can show soft tissue mineralization (calcification) in these tumors 
    • may resemble heterotopic ossification
  • CT
    • can show calcification in the soft tissue mass 
  • MRI
    • MRI reveals a heterogenous mass that is typically dark on T1 weighted images and bright on T2 weighted images
Histology
  • Characteristic findings 
    • classical synovial sarcoma shows a biphasic appearance with two typical cell types
      • spindle cells (fibrous type of cells) 
        • relatively small and uniform and found in sheets of malignant appearing cells with minimal cytoplasm and dark atypical nuclei
      • epithelial cells 
        • gland, nest, or cyst like cells    
    • rarely, synovial sarcoma can also present with poorly differentiated or monophasic fibrous cell histology, consisting only of sheets of spindle cells
    • cellular origin of synovial sarcoma is unknown, but it is not the synovial cell or any cell involved in the synovium.  The name SYNOVIAL sarcoma is a misnomer  
  • Immunostaining for   
    • synovial sarcoma stains positive for 
      • vimentin 
      • epithelial membrane antigen
      • sporadic S-100 
      • epithelial cells stain positive for keratin
Treatment
  • Operative
    • wide surgical resection with adjuvant radiotherapy
      • indications
        • standard of care in most patients
      • technique
        • radiotherapy
          • may be delivered either pre-surgery or post-surgery
        • chemotherapy
          • data regarding chemotherapy in synovial sarcoma suggests that chemotherapy may improve both local control and overall survival
IBank
  Location
Xray
Xray
CT
B. Scan
MRI
MRI
Histo(1)
Case A
pelvis
 
 
 
Case B
axilla
 
 
 
Case C
leg
 
 
 
 
Case D
             
(1) - histology does not always correspond to clinical case 



 

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Qbank (9 Questions)

TAG
(OBQ13.17) Which of the following tumors associated with the fusion protein SYT-SSX1 can metastasize via the lymph nodes? Topic Review Topic

1. Alveolar rhabdomyosarcoma
2. Epitheliod sarcoma
3. Clear cell sarcoma
4. Angiosarcoma
5. Synovial sarcoma

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TAG
(OBQ11.98) Which of the following fusion proteins is associated with synovial sarcoma? Topic Review Topic

1. BCR-ABL
2. SYT-SSX
3. EWS-FLI1
4. FUS-CHOP
5. COL1A1-PDGFB

PREFERRED RESPONSE ▶
TAG
(OBQ08.15) A 22-year-old female has a painful foot mass. Clinical photo, MRI, and histology slide are shown in Figures A through C. What is the most likely diagnosis? Topic Review Topic
FIGURES: A   B   C      

1. Osteosarcoma
2. Morton's neuroma
3. Synovial sarcoma
4. Fibrous dysplasia
5. Desmoid tumor

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TAG
(OBQ08.236) All of the following statements regarding synovial sarcoma are correct EXCEPT? Topic Review Topic

1. Typically occur around the joints of the arm or leg
2. Immunohistochemisty is positive for cytokeratin and epithelial membrane antigen
3. Histology is named because the cell line of origin is the type II synovial cell
4. May show biphasic or monophasic histology
5. Classic age range is the younger adult < 40 years of age

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TAG
(OBQ05.16) A 33-year-old male presents with a painful mass in his axilla. Radiographs, MRI, and histology are shown in Figures A-C. The tumor stains positive for vimentin and epithelial membrane antigen on immunohistochemistry. What is the most likely diagnosis? Topic Review Topic
FIGURES: A   B   C      

1. Synovial sarcoma
2. Liposarcoma
3. Malignant fibrous histiocytoma
4. Osteosarcoma
5. Chondrosarcoma

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TAG
(OBQ05.72) Which of the following is the most common sarcoma found in the foot? Topic Review Topic

1. Epithelioid Sarcoma
2. Liposarcoma
3. Fibrosarcoma
4. Synovial sarcoma
5. Adamantinoma

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TAG
(OBQ05.253) The chromosomal translocation t(X;18)is found in which of the following conditions? Topic Review Topic

1. Myxoid liposarcoma
2. Synovial Sarcoma
3. Osteosarcoma
4. Ewing's Sarcoma
5. Clear cell sarcoma

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TAG
(OBQ04.172) Which of the following histology images represents a synovial sarcoma? Topic Review Topic
FIGURES: A   B   C   D   E  

1. Figure A
2. Figure B
3. Figure C
4. Figure D
5. Figure E

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