Ectomesenchymal Chondromyxoid Tumor: A Rare Case Report With an 11-Year Indolent Course.

Ectomesenchymal chondromyxoid tumor (EMCMT) is a rare benign neoplasm of uncertain histogenesis that typically involves the anterior portion of the tongue. Approximately 114 cases have been documented in the literature, with 9.6% reported from South America. Herein, we describe a case of EMCMT and discuss its clinicopathological features, differential diagnosis, and treatment. A 25-year-old woman presented with an 11-year history of an asymptomatic nodular lesion on the left lateral-middle dorsal surface of the tongue. Surgical excision was performed. Histopathological examination revealed a multilobulated myxoid tumor composed of spindle-shaped and polygonal cells. Immunohistochemical analysis showed diffuse positivity for glial fibrillary acidic protein and S-100 protein. No recurrence was observed after 12 months of follow-up. This case highlights the prototypical clinicopathological features of EMCMT and underscores the importance of its recognition in the differential diagnosis of tongue nodules. To the best of our knowledge, this represents the first documented case from Venezuela, contributing to the geographic expansion of EMCMT data and emphasizing the need for clinical awareness, particularly in resource-limited settings.