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 22 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 Previous Next 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? Tested Concept QID: 4652 Type & Select Correct Answer 1 Alveolar rhabdomyosarcoma 3% (124/4102) 2 Epitheliod sarcoma 4% (163/4102) 3 Clear cell sarcoma 5% (196/4102) 4 Angiosarcoma 3% (103/4102) 5 Synovial sarcoma 85% (3493/4102) L 2 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 5 Review tested concept (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? Tested Concept QID: 4815 FIGURES: A B C D Type & Select Correct Answer 1 Synovial sarcoma 77% (2278/2960) 2 Liposarcoma 5% (143/2960) 3 Angiosarcoma 3% (89/2960) 4 Schwannoma 8% (232/2960) 5 Rhabdomyosarcoma 7% (201/2960) L 3 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review tested concept Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ11.98) Which of the following fusion proteins is associated with synovial sarcoma? Tested Concept QID: 3521 Type & Select Correct Answer 1 BCR-ABL 1% (25/2292) 2 SYT-SSX 90% (2070/2292) 3 EWS-FLI1 3% (64/2292) 4 FUS-CHOP 2% (47/2292) 5 COL1A1-PDGFB 3% (73/2292) L 1 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review tested concept 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 (OBQ08.236) All of the following statements regarding synovial sarcoma are correct EXCEPT? Tested Concept QID: 622 Type & Select Correct Answer 1 Typically occur around the joints of the upper or lower limb 10% (75/725) 2 Immunohistochemisty is positive for cytokeratin and epithelial membrane antigen 10% (70/725) 3 Histology is named because the cell line of origin is the type II synovial cell 62% (450/725) 4 May show biphasic or monophasic histology 7% (52/725) 5 Classic age range is the younger adult < 40 years of age 10% (70/725) L 3 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review tested concept (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? Tested Concept QID: 401 FIGURES: A B C Type & Select Correct Answer 1 Osteosarcoma 1% (21/2457) 2 Morton's neuroma 2% (55/2457) 3 Synovial sarcoma 60% (1484/2457) 4 Fibrous dysplasia 5% (118/2457) 5 Desmoid tumor 31% (771/2457) L 3 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 3 Review tested concept Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK Sorry, this question is for PEAK Premium Subscribers only Upgrade to PEAK (OBQ07.104) The fusion gene resulting from the chromosomal translocation t(X;18)(p11;q11) is chararcteristically found in which malignancy? Tested Concept QID: 765 Type & Select Correct Answer 1 Synovial sarcoma 80% (946/1183) 2 Chronic myelogenous leukemia (CML) 3% (30/1183) 3 Ewings sarcoma 11% (126/1183) 4 Alveolar rhabdomyosarcoma 3% (39/1183) 5 Clear cell sarcoma 3% (40/1183) L 2 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review tested concept (OBQ05.72) Which of the following is the most common sarcoma found in the foot? Tested Concept QID: 958 Type & Select Correct Answer 1 Epithelioid Sarcoma 12% (344/2959) 2 Liposarcoma 2% (66/2959) 3 Fibrosarcoma 8% (233/2959) 4 Synovial sarcoma 77% (2274/2959) 5 Adamantinoma 1% (29/2959) L 2 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 4 Review tested concept (OBQ05.253) The chromosomal translocation t(X;18)is found in which of the following conditions? Tested Concept QID: 1139 Type & Select Correct Answer 1 Myxoid liposarcoma 4% (64/1718) 2 Synovial Sarcoma 83% (1422/1718) 3 Osteosarcoma 3% (47/1718) 4 Ewing's Sarcoma 7% (112/1718) 5 Clear cell sarcoma 4% (64/1718) L 2 Question Complexity B Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 2 Review tested concept (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? Tested Concept QID: 53 FIGURES: A B C Type & Select Correct Answer 1 Synovial sarcoma 65% (807/1248) 2 Liposarcoma 10% (128/1248) 3 Malignant fibrous histiocytoma 22% (275/1248) 4 Osteosarcoma 1% (7/1248) 5 Chondrosarcoma 2% (26/1248) L 3 Question Complexity C Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review tested concept (OBQ04.172) Which of the following histology images represents a synovial sarcoma? Tested Concept QID: 1277 FIGURES: A B C D E Type & Select Correct Answer 1 Figure A 63% (719/1134) 2 Figure B 2% (27/1134) 3 Figure C 14% (164/1134) 4 Figure D 8% (92/1134) 5 Figure E 11% (127/1134) L 3 Question Complexity D Question Importance Select Answer to see Preferred Response SUBMIT RESPONSE 1 Review tested concept
All Videos (0) Podcasts (2) Question Session⎪Synovial Sarcoma & Humeral Shaft Fractures Orthobullets Team Pathology - Synovial Sarcoma Listen Now 23:9 min 11/8/2019 18 plays (0) Pathology⎪Synovial Sarcoma Team Orthobullets 4 Pathology - Synovial Sarcoma Listen Now 5:52 min 10/16/2019 88 plays (2)