Synovial sarcoma of the larynx, a rare and unusual entity. Case report.

Abstract

Introduction and importance: Synovial sarcoma (SS) is a high-grade malignancy, accounting for 2.5-3.5 % of all primary H&N sarcomas and 0.1 % of all H&N cancers.

Case presentation: A 26-year-old woman presents with dyspnea and dysphonia. Flexible nasofibrolaryngoscopy and CT were performed with evidence of a laryngeal tumor. Total laryngectomy was performed due to a histopathological report of laryngeal synovial sarcoma.

Clinical discussion: Primary SS of the H&N is rare and accounts for <5 % of all synovial sarcomas and laryngeal-origin SS represents a rare subset within H&N sarcomas. There are 3 different histologic subtypes: biphasic, monophasic and poorly differentiated. Immunohistochemistry plays a crucial role in histologic diagnosis. The optimal treatment of synovial sarcoma is multimodal, depending on its size, location and grade. Radical surgical excision with negative margins is generally accepted as the mainstay of treatment. Disease recurrence is a significant problem, with up to 45 % of patients developing local disease and 33 % developing metastatic disease.

Conclusions: Laryngeal SS is exceptionally rare, requiring complex, multidisciplinary management.