Ectomesenchymal chondromyxoid tumor: Review of literature and a report of a rare case

Abstract

Ectomesenchymal chondromyxoid tumor (ECMT) is a rare benign intraoral tumor. Clinically, it presents as a slow growing, painless, firm, submucosal sweling exclusively occurring on the anterior dorsum of the tongue. Till date only 40 cases have been reported in literature. Histopathologically the tumor is characterized by a well circumscribed, lobular proliferation of round, polygonal, ovoid or fusiform cells in a net-like pattern in a myxoid to chondromyxoid background. Here, we present a rare case of ECMT occurring in a 7-year-old boy and throw some light on this distinct entity.

Ectomesenchymal Chondromyxoid Tumor: A Series of Seven Cases and Review of the Literature

“…Our study shows that ECTs arise in a wide age range (7-57 years) with a slight tendency to affect males; however, literature review shows no sex preference [3]. In our series, six out of seven tumors were located on the anterior dorsal tongue, which is consistent with previously reported cases.…”

“…Despite the original description of ECT, the clinical, histopathologic and immunohistochemical features of this tumor have not been thoroughly defined, as these are rare tumors, with only 48 ECTs reported in the English language literature. Some authors attribute the low number of diagnosed cases to the possibility of confusion with other entities that may share some histopathological features, such as myoepithelioma, oral focal mucinosis, soft tissue myxoma, ossifying fibromyxoid tumor, chondroid choristoma, nerve sheath myxoma, pleomorphic adenoma, and mucocele [2,3].…”

“…The cells are either round, oval, polygonal or spindled in morphology. They may have multilobulated nuclei and may occasionally show atypia [1][2][3].…”

Ectomesenchymal chondromyxoid tumor: a comprehensive updated review of the literature and case report

“…Of them, 45 were located on the anterior aspect of the tongue and ranged in size from 3 to 50 mm (mean 13.3 mm) 1 – 3 , 5 – 7 , 9 – 24 , 26 , 28 , 30 , 31 ; the two lesions on the posterior tongue each had a diameter of 20 mm. 4 , 16 In the 31 cases (exclusively anterior lesions) that described the impression of palpation, 19 cases were described as firm 1 , 2 , 5 , 7 , 9 , 10 , 12 – 14 , 16 , 17 , 20 , 23 , 26 , 31 , 1 as soft 1 , 1 cystic, 1 1 firm-cystic 1 , 4 elastic, 3 , 18 , 19 , 22 2 non-tender 24 , 28 , 1 tender 20 , 1 firm-cystic 1 , 1 soft-cystic. 6 …”

Ectomesenchymal Chondromyxoid Tumour of the Dorsal Tongue Presenting with Impaired Speech

“…However, this lack of definition of ECT's features likely also results from it being confused with or mistaken for other entities that share some of its histopathological features. Entities to include in the differential diagnosis include myoepithelioma, nerve sheath myxoma, mucocele, pleomorphic adenoma, oral focal mucinosis, glial choristoma, ossifying fibromyxoid tumour, chondroid choristoma, soft tissue myxoma, and cellular neurothekeoma [2, 10, 11]. The development and refinement of immunohistochemistry studies, and the identification of specific molecular markers, have allowed pathologists to distinguish ECT from other entities in the differential diagnosis.…”