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
QUESTIONS 1 of 19 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 Previous Next (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: A B C D 1 Synovial sarcoma 77% (2031/2648) 2 Liposarcoma 5% (131/2648) 3 Angiosarcoma 3% (78/2648) 4 Schwannoma 8% (212/2648) 5 Rhabdomyosarcoma 7% (179/2648) ML 3 Select Answer to see Preferred Response PREFERRED RESPONSE 1 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (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% (111/3720) 2 Epitheliod sarcoma 4% (146/3720) 3 Clear cell sarcoma 5% (175/3720) 4 Angiosarcoma 2% (89/3720) 5 Synovial sarcoma 86% (3181/3720) ML 2 Select Answer to see Preferred Response PREFERRED RESPONSE 5 (OBQ04.172) Which of the following histology images represents a synovial sarcoma? Review Topic QID: 1277 FIGURES: A B C D E 1 Figure A 66% (614/925) 2 Figure B 2% (14/925) 3 Figure C 14% (132/925) 4 Figure D 6% (59/925) 5 Figure E 11% (102/925) ML 3 Select Answer to see Preferred Response 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% (304/2615) 2 Liposarcoma 2% (57/2615) 3 Fibrosarcoma 8% (218/2615) 4 Synovial sarcoma 76% (1999/2615) 5 Adamantinoma 1% (25/2615) ML 2 Select Answer to see Preferred Response 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 Select Answer to see Preferred Response 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 Select Answer to see Preferred Response 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% (61/553) 2 Immunohistochemisty is positive for cytokeratin and epithelial membrane antigen 10% (54/553) 3 Histology is named because the cell line of origin is the type II synovial cell 61% (335/553) 4 May show biphasic or monophasic histology 8% (44/553) 5 Classic age range is the younger adult < 40 years of age 9% (52/553) ML 3 Select Answer to see Preferred Response PREFERRED RESPONSE 3 (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: A B C 1 Synovial sarcoma 65% (705/1089) 2 Liposarcoma 9% (102/1089) 3 Malignant fibrous histiocytoma 23% (250/1089) 4 Osteosarcoma 1% (7/1089) 5 Chondrosarcoma 2% (20/1089) ML 3 Select Answer to see Preferred Response 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: A B C 1 Osteosarcoma 1% (20/2186) 2 Morton's neuroma 2% (42/2186) 3 Synovial sarcoma 60% (1320/2186) 4 Fibrous dysplasia 4% (96/2186) 5 Desmoid tumor 32% (701/2186) ML 3 Select Answer to see Preferred Response PREFERRED RESPONSE 3 Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK