Ectomesenchymal chondromyxoid tumor: A rare case report

Ectomesenchymal chondromyxoid tumor (ECMT) is a rare benign neoplasm of uncertain histogenesis, which exclusively involves the oral cavity, particularly the tongue. Clinically, it presents as slow growing, painless, firm, submucosal swelling exclusively occurring on the anterior dorsum of the tongue. Histopathologically, it comprised well circumscribed, unencapsulated lobular proliferation of fusiform and polygonal cells, with varying degree of cellularity, with neoplastic cells often seen in a myxoid, chondroid or hyalinized background. Until date, only 40 cases have been reported in the literature. Most documented lesions involve anterior tongue, however 2 cases in posterior tongue and one palatal tumor has been described. Here, we present a rare case diagnosed clinically and histopathologically as (ECMT) in a 7-year-old girl with the size of the lesion 5.0 cm making this case even rarer and throwing some light on this distinct entity.