Abstract
Primary synovial sarcoma of larynx: Clinicopathologic features of an enigmatic entity posing diagnostic and therapeutic challenges
Highlights
Synovial sarcoma is an aggressive, yet a relatively chemosensitive malignant mesenchymal neoplasm which displays variable epithelial differentiation on histopathologic examination, and typically occurs in adolescents and young adults, commonly in males [13]
We report three cases of primary laryngeal synovial sarcoma which presented a diagnostic challenge and were confirmed by molecular testing
We describe the clinical, histopathologic and molecular features of primary laryngeal synovial sarcomas diagnosed at our tertiary care centre over a period of eight years, and review cases previously published in literature (Table 1)
Summary
Synovial sarcoma is an aggressive, yet a relatively chemosensitive malignant mesenchymal neoplasm which displays variable epithelial differentiation on histopathologic examination, and typically occurs in adolescents and young adults, commonly in males [13]. Synovial sarcoma is characterized by a unique molecular signature, t(x;18) (p11.2; q11.2) translocation, resulting in the fusion of SS18 (formerly SYT) gene with one of the SSX genes (SSX1, SSX2, and rarely, SSX4) [5]. Identification of this translocation by fluorescence in situ hybridization or reverse-transcriptase polymerase chain reaction is accepted as the diagnostic standard, irrespective of morphologic features and tumor location [3]. The larynx is an extremely rare site, accounting for 3-10% of all head and neck synovial sarcomas [4].
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