Synovial sarcoma overview

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Synovial sarcoma is a rare soft-tissue sarcoma defined by the presence of a unique translocation, t(X;18)(q11.2;p11.2). It usually occurs adjacent to joints in the limbs and was originally thought to arise from synovium.

However, synovial sarcoma is a misnomer and the origin of the tissue is unknown. Primary synovial sarcomas have been documented in most human tissues and organs, including the brain, prostate and heart. Synovial sarcoma occurs most commonly in adolescents and young adults. Diagnosis is based on histology; there are three major histologic subtypes: monophasic (consisting on sheets of spindle cells), biphasic (spindle cells admixed with areas of epithelial differentiation) and poorly differentiated. Immunohistochemical techniques can help, however the gold standard for diagnosing synovial sarcoma is detection of t(X;18) by cytogenetics, FISH or RT-PCR. Despite treatment, prognosis is generally poor, with approximately 50% of patients dying from local reccurrence or metastasis within 10 years.

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