AIMS: To evaluate diagnostic accuracy of SSX and SSX-SS18 antibodies in decalcified surgical specimens and outcome of synovial sarcomas (SS) of bone.
METHODS AND RESULTS: Twenty-five cases were classified as bone SS (prevalence 0.32% among malignant primary bone sarcoma). Median age was 34 years (range 9 -79) . 24/25 patients presented with non-metastatic tumors, one with lung metastases. The majority of tumors involved the long bones of extremities with metaphyseal origin. Mean size of the tumor was 7.1 cm. 20 cases (80%) were monophasic and 5 (20%) biphasic. SS18-SSX fusion-specific antibody had 92% sensitivity and 99% specificity for primary bone SS, whereas SSX C-terminus antibody had 100% sensitivity and 94% specificity. FISH analysis was feasible in 9 (36%) cases and detected SS18 rearrangement in all 9 cases. All patients underwent surgical removal of their primary tumor, with adequate margins in 18 (72%) patients. Chemotherapy with metothrexate, cisplatin, doxorubicin, ifosfamide was used in the 7 patients. Two patients with inadequate surgical margins received radiotherapy. With a median follow-up of 80 months (range 6-428), 5-year and 10-year overall survival (OS) were 66.6% and 47.9% respectively and 5-and 10-years disease-free survival (DFS) were 36.8% (95% C.I.: 18.0-55.7%), and 32.2% (95% C.I.: 14.6-51.2%) respectively A significant improvement in 10-years DFS in patients undergoing chemotherapy as compared with patients who did not was observed (p 0.039).
CONCLUSIONS: Our series highlights the utility of SS18-SSX fusion-specific and SSX C-terminus antibodies to support the diagnosis of SS, Adjustment chemotherapy was associated with improved prognosis in this series.
- adoloscent and young adults
- bone tumors
- synovial sarcoma
- ultra rare bone sarcoma