This report outlines the workup and management of a 55-year-old woman with a synovial sarcoma of the lateral border of the tongue that was initially diagnosed as a glomus tumor. A review was performed of the literature on synovial sarcomas of the oral cavity and current National Comprehensive Cancer Network guidelines. Synovial sarcomas of the tongue are rare neoplasms, with variable morphologic microscopic types and immunohistochemical profiles. Fluorescence in situ hybridization analysis of the known gene translocation also can be used in diagnosis. According to the literature, resection of the tumor is the current treatment of choice; however, owing to the rarity of this entity, diagnosis and management prove challenging for the oral and maxillofacial surgeon.
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