Abstract: Ectomesenchymal chondromyxoid tumor is a rare benign intraoral soft tissue neoplasm. Till date, 93 cases have been reported in literature. Clinically, it presents as a slowly growing, painless, firm, submucosal swelling mainly occurring on the anterior two thirds of the dorsum of the tongue. Histopathologically, the tumor is characterized by a well circumscribed, lobular proliferation of round, polygonal, or fusiform cells in a net-like pattern in a myxoid to chondromyxoid background.

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... Although it shows a morphological resemblance to myoepitheliomas of soft tissue and skin, the exact histogenesis remains unclear; a recent publication of a large series has demonstrated recurrent RREB1-MKL2 gene fusions in 90% of the cases, suggesting the possibility of a new entity. The diagnosis is largely supported by immunhistochemical investigations. Here, we present a new case of ECT occurring in a 62-year-old male who presented with a leading complaint of slowly growing painless mass arising from the anterior part of the tongue measuring about 3 cm. This lesion was diagnosed 4 years before recent admittance, but the patient did not undergo the scheduled surgical intervention and was lost to follow-up. As the tumor was still persisting and moderately enlarging, he came back to our clinic. Finally, the mass was completely resected; after 24 month of follow-up, he is doing well, with no signs of recurrence. Orv Hetil. 2019; 160(33): 1319–1323.