Updated: 6/3/2019

Synovial Sarcoma

Topic
Review Topic
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Questions
19
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Evidence
12
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https://upload.orthobullets.com/topic/8052/images/Case B - axilla - xray - parsons_moved.jpg
https://upload.orthobullets.com/topic/8052/images/Case B - axilla - MRI - parsons_moved.jpg
https://upload.orthobullets.com/topic/8052/images/Histology B - Parsons_moved.png
https://upload.orthobullets.com/topic/8052/images/Case A - pelvis - xray - parsons_moved.jpg
https://upload.orthobullets.com/topic/8052/images/Case A - pelvis - CT - parsons_moved.png
https://upload.orthobullets.com/topic/8052/images/synovial-sarcoma-25-sarcoma-images_moved.jpg
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 
  • Pathophysiology
    • metastasis
      • 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 metastasize 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
  • Genetics
    • chromosomal translocation t(X;18) is observed in more than 90% of cases   
    • translocation forms the SYT-SSX1, 2, or 4 fusion protein  
      • SYT-SSX4 is rare
  • Prognosis
    • overall prognosis is poor
      • 5 year survival is approximately 50%
      • 10 year survival is approximately 25%
    • SYT-SSX fusion type is most important prognostic factor
      • SYT-SSX2 better survival
  SYT-SSX1  SYT-SSX2
Frequency More common (60% of tumors) Less common (40% of tumors)
Histology type
Biphasic 
Monophasic
Gender
M:F = 1:1
M:F = 1:2
Presentation Larger, with metastases Smaller, without metastases
Survival Worse Better
 
Presentation
  • Symptoms
    • typically present as a growing mass in proximity to a joint
    • may be painless or painful
    • most commonly occur in periarticular locations
      • knee, shoulder, elbow, foot
      • 60% are found in the lower extremity
  • Physical exam
    • check 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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Questions (19)

(OBQ13.180) A 29-year-old male presents with deep pain and swelling of the right foot. He first noticed pain and swelling 1 year ago, which has been steadily increasing over this time. He denies any recent trauma, travel or systemic symptoms. A radiograph of the foot is shown in Figure A. Figures B and C show images of an MRI scan of the right foot. A biopsy was performed as shown in Figure D. What is the most likely diagnosis? Review Topic

QID: 4815
FIGURES:
1

Synovial sarcoma

77%

(2037/2656)

2

Liposarcoma

5%

(131/2656)

3

Angiosarcoma

3%

(79/2656)

4

Schwannoma

8%

(212/2656)

5

Rhabdomyosarcoma

7%

(180/2656)

ML 3

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PREFERRED RESPONSE 1
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(OBQ13.17) Which of the following tumors associated with the fusion protein SYT-SSX1 can metastasize via the lymph nodes?
Review Topic

QID: 4652
1

Alveolar rhabdomyosarcoma

3%

(111/3733)

2

Epitheliod sarcoma

4%

(147/3733)

3

Clear cell sarcoma

5%

(176/3733)

4

Angiosarcoma

2%

(90/3733)

5

Synovial sarcoma

85%

(3191/3733)

ML 2

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PREFERRED RESPONSE 5

(OBQ04.172) Which of the following histology images represents a synovial sarcoma? Review Topic

QID: 1277
FIGURES:
1

Figure A

66%

(613/926)

2

Figure B

2%

(14/926)

3

Figure C

14%

(133/926)

4

Figure D

6%

(59/926)

5

Figure E

11%

(103/926)

ML 3

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PREFERRED RESPONSE 1

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

QID: 958
1

Epithelioid Sarcoma

12%

(304/2629)

2

Liposarcoma

2%

(57/2629)

3

Fibrosarcoma

8%

(219/2629)

4

Synovial sarcoma

77%

(2012/2629)

5

Adamantinoma

1%

(25/2629)

ML 2

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PREFERRED RESPONSE 4

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

QID: 1139
1

Myxoid liposarcoma

4%

(53/1504)

2

Synovial Sarcoma

84%

(1261/1504)

3

Osteosarcoma

2%

(37/1504)

4

Ewing's Sarcoma

6%

(95/1504)

5

Clear cell sarcoma

3%

(51/1504)

ML 2

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PREFERRED RESPONSE 2

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

QID: 3521
1

BCR-ABL

1%

(23/2065)

2

SYT-SSX

90%

(1868/2065)

3

EWS-FLI1

3%

(57/2065)

4

FUS-CHOP

2%

(38/2065)

5

COL1A1-PDGFB

3%

(68/2065)

ML 1

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PREFERRED RESPONSE 2

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

QID: 622
1

Typically occur around the joints of the upper or lower limb

11%

(60/551)

2

Immunohistochemisty is positive for cytokeratin and epithelial membrane antigen

10%

(54/551)

3

Histology is named because the cell line of origin is the type II synovial cell

61%

(334/551)

4

May show biphasic or monophasic histology

8%

(44/551)

5

Classic age range is the younger adult < 40 years of age

9%

(52/551)

ML 3

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(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? Review Topic

QID: 53
FIGURES:
1

Synovial sarcoma

65%

(703/1087)

2

Liposarcoma

9%

(102/1087)

3

Malignant fibrous histiocytoma

23%

(250/1087)

4

Osteosarcoma

1%

(7/1087)

5

Chondrosarcoma

2%

(20/1087)

ML 3

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PREFERRED RESPONSE 1

(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? Review Topic

QID: 401
FIGURES:
1

Osteosarcoma

1%

(20/2197)

2

Morton's neuroma

2%

(42/2197)

3

Synovial sarcoma

61%

(1330/2197)

4

Fibrous dysplasia

4%

(96/2197)

5

Desmoid tumor

32%

(702/2197)

ML 3

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PREFERRED RESPONSE 3
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