Synovial sarcomas - Epidemiologic and histologic features, prognostic factors and treatment

Underestimated frequency: Synovial sarcoma is the third most common histologic type of soft tissue sarcoma in the limbs. Synovial sarcoma generally occurs in two distinct histologic subtypes. The biphasic type contains both epithelial and spindle cell components, whereas the monophasic fibrous type is a spindle cell tumor with no epithelial component Pathology: Immunostaining for keratin and vimetine and the presence of the specific translocation t(X;18)(p11.2;q11.2) may prove extremely useful diagnostic markers. Prognosis:Favorable prognostic factors are tumor size, low mitotic activity (less than 10 to 15 mitoses per high-power field), microscopic dean margins of resection. Management: There are few specific data on specific treatment for synovial sarcoma. In retrospective studies, systemic chemotherapy is based on doxorubicin-containing regimens or on ifosfamide used as a single agent. In fact, like other soft tissue sarcomas and despite the lack of any proven effect on survival, adjuvant chemotherapy with doxorubicin alone or combined with ifosfamide is most commonly used before surgery. When necessary, radiation therapy in combination with surgery is generally employed in the postoperative period. The association of preoperative chemotherapy, surgical resection and post operative radiotherapy allows limb-sparing surgical procedures with the same overall survival as with amputation.