Primary synovial sarcoma of larynx: Clinicopathologic features of an enigmatic entity posing diagnostic and therapeutic challenges

Synovial Sarcoma (SS) is an aggressive, yet a relatively chemosensitive malignant mesenchymal neoplasm, which displays variable epithelial differentiation, and typically occurs in adolescents and young adults, most frequently in the lower extremities. SS is also reported at certain unconventional sites, where its inherent morphologic heterogeneity and overlapping histopathologic features with certain tumors indigenous to that site can lead to a diagnostic dilemma. Presently, the diagnostic gold standard of a synovial sarcoma is demonstration of the characteristic t(X;18) (p11.2; q11.2) translocation, resulting in SYT-SSX fusion, especially in cases occurring at unconventional sites. Larynx is one of the rarely reported sites for a synovial sarcoma, with only 28 such cases reported till date, including only six cases confirmed by molecular testing. We report three cases of primary laryngeal synovial sarcoma which presented a diagnostic challenge and were confirmed by molecular testing. Although rare, SYNOVIAL SARCOMA should be kept as a differential diagnosis of spindle cell neoplasms and biphasic tumors occurring in the larynx. In view of the difference in treatment and patient outcome from the relatively more frequently occurring tumors in the larynx, such as sarcomatoid carcinoma and minor salivary gland neoplasms, its accurate diagnosis with molecular testing in such cases is crucial. Correspondence to: Dr. Shubhada V Kane, Professor and Head, Department of Pathology, Tata Memorial Hospital, Mumbai, India-400012; Tel: +919223424134; Fax: +91-22-24146937; E-mail: [email protected]