Synovial Sarcoma

Abstract

Synovial sarcomas are a common extremity soft tissue sarcoma, typically arising in the para-articular regions of young adults. All synovial sarcomas are considered high grade. Distinctive clinical features are the two common histologic variants (monophasic and biphasic) and more frequent incidence of nodal metastasis. In addition to the sheets of spindle cells seen in monophasic tumors, biphasic tumors have interspersed areas of glandular differentiation. Synovial sarcomas are characterized by the SS18-SSX translocation. Most biphasic tumors contain the SS18-SSX1 translocation, whereas almost all SS18-SSX2 tumors are monophasic. Radical resection is the mainstay of therapy. Synovial sarcomas are chemosensitive, and ifosfamide-doxorubicin is often used neoadjuvantly. Adjuvant radiation should be considered for tumors greater than 5 cm. This review contains 7 figures, 2 tables, and 35 references. Key Words: E-cadherin, extremity sarcoma, ifosfamide, neoadjuvant chemotherapy, nomogram, SS18-SSX fusion, synovial sarcoma, translocation-associated sarcoma