Ectomesenchymal chondromyxoid tumor (ECMT) is a rare benign neoplasm arising in the tongue. With only 45 cases reported in the literature, there are several unique features defining this lesion. Firstly, almost all patients present with an asymptomatic slow growing mass on the anterior dorsum of the tongue. At the microscopic level, it is recognizable as a well-circumscribed unencapsulated proliferation of uniform round to fusiform cells embedded in a chondromyxoid matrix. Lastly, the immunohistochemistry profile is characterised by positivity for glial fibrillary acidic protein and frequent positivity for S-100 and cytokeratins. We report a case of a mass located on the posterior dorsum of the tongue and meeting the aforementioned morphological and immunohistochemical criteria of ECMT.
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