Abstract
 Ectomesenchymal chondromyxoid tumor (ECT), first reported by Smith et al. in 1995, is an extremely rare benign tumor mostly occurring on the dorsal surface of the tongue. ECT shows histopathologically similar findings to salivary gland tumors, and its histological origin is still under debate. We report a case of ECT arising in the tongue in a 31-year-old man. A dark purple, soft-firm mass was found on the anterior portion of the left lingual border. The tumor was resected along the extracapsular surface under general anesthesia. The resection showed a multi-lobular tumor surrounded by hemorrhage. Histopathologically, polygonal tumor cells proliferated in a reticular pattern in a myxoid background. The basement membrane, which is always present in salivary gland tumors, was not seen. Immunohistochemistry showed that the tumor cells were positive for GFAP, vimentin, S-100 protein and smooth muscle actin, but negative for cytokeratin (AE1/AE3), basal cell marker p63, and epithelial membrane antigen. Based on these histopathological and immunohistochemical findings, the tumor was diagnosed as ECT. The patient has a good postoperative course and shows no signs of recurrence.