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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\u2010year\u2010old woman presented with an 11\u2010year history of an asymptomatic nodular lesion on the left lateral\u2010middle dorsal surface of the tongue. Surgical excision was performed. Histopathological examination revealed a multilobulated myxoid tumor composed of spindle\u2010shaped and polygonal cells. Immunohistochemical analysis showed diffuse positivity for glial fibrillary acidic protein and S\u2010100 protein. No recurrence was observed after 12\u2009months of follow\u2010up. 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\u2010limited settings.<\/jats:p>","DOI":"10.1111\/cup.70021","type":"journal-article","created":{"date-parts":[[2025,11,25]],"date-time":"2025-11-25T02:04:55Z","timestamp":1764036295000},"page":"219-223","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Ectomesenchymal Chondromyxoid Tumor: A Rare Case Report With an 11\u2010Year Indolent Course"],"prefix":"10.1111","volume":"53","author":[{"given":"Mariana","family":"Villarroel\u2010Dorrego","sequence":"first","affiliation":[{"name":"School of Dentistry Universidad Central de Venezuela  Caracas Venezuela"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria 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