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

Department of Oral and Maxillofacial Pathology, The Oxford Dental College, Hospital and Research Centre, Bommanahalli, Hosur Road, Bangalore, Karnataka, India.
Journal of Oral and Maxillofacial Pathology 04/2011; 15(1):74-9. DOI: 10.4103/0973-029X.80021
Source: PubMed


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.

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    ABSTRACT: Ectomesenchymal chondromyxoid tumor (ECMT) is a rare entity of the dorsal tongue first described in 1995. Herein, we report a rare case of lingual ECMT in a 41-year-old man. Patient presented with an asymptomatic, small nodule (0.5 cm in diameter) in the anterior tongue. The pathological findings showed uni-lobular proliferation of fusiform cells, arranged in net-like sheets or swirls, in a chondromyxoid background. The tumor cells were immunoreactive for S-100 and glial fibrillary acidic protein (GFAP), but negative for epithelial markers. Familiarity with this entity helps pathologists make a correct diagnosis.
    Indian Journal of Pathology and Microbiology 10/2012; 55(4):519-20. DOI:10.4103/0377-4929.107796 · 0.47 Impact Factor
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    ABSTRACT: The objective of this study was to investigate the histogenesis of ectomesenchymal chondromyxoid tumors (ECTs) of the tongue. The biochemical characteristics of a rarely occurring tumor of the tongue were analyzed by immunohistochemistry and reverse-transcriptase polymerase chain reaction (RT-PCR), and its biological properties were assessed in primary culture in serum-free media. Immunohistochemistry showed that the tumor cells were strongly positive for vimentin, S-100, and glial fibrillary acidic protein (GFAP), but negative for cytokeratin and epithelial membrane antigen. In primary cultures, the cells derived from the ECT were morphologically similar to neuronal cells and expressed Nanog, GFAP, and MAP2. RT-PCR analysis of the surgical specimen was positive for OCT3/4, Sox2, Nanog, MAP2, and CD105 mRNAs. The results of the present study indicate that ECTs originate from the ectomesenchymal cells of the neural crest and are similar in their molecular and biological characteristics to undifferentiated mesenchymal stem cells.
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