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

International Journal of Oral Science
Astrid TruschneggAlfred Beham

Abstract

Prompted by a unique case of an ectomesenchymal chondromyxoid tumor (ECT) of the palate in a 54-year-old female, we reviewed the English and German literature on this entity until the end of 2016 using PubMed. The search produced 74 lingual cases with a nearly equal sex distribution and a mean age of 39.3 years, and two extra-lingual cases sharing histological and immunohistological features including nodular growth, round, fusiform or spindle-shaped cellular architecture, and chondromyxoid stroma. Immunophenotyping showed the majority of cases to be positive for glial fibrillary acidic protein (GFAP), S-100 protein, glycoprotein CD57, pancytokeratin (AE1/AE3), and smooth muscle actin (SMA); in isolated cases there was molecular-genetic rearrangement or gain of Ewing sarcoma breakpoint region 1 (EWSR1) but no rearrangement of pleomorphic adenoma gene 1 (PLAG1). At present, ectomesenchymal cells that migrate from the neural crest are considered to play a pivotal role in tumor origin. All cases had a benign course, although there were three recurrences. Because of the rarity of this tumor and the need for differential diagnostic differentiation from myoepithelioma and pleomorphic adenoma, both oral surgeons and pathologists shoul...Continue Reading

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Citations

Oct 28, 2019·BMJ Case Reports·Jeyashanth RijuRamesh Babu Telugu
Oct 6, 2020·Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology·Junho JungJoo-Young Ohe
Aug 31, 2021·The Cleft Palate-craniofacial Journal : Official Publication of the American Cleft Palate-Craniofacial Association·Sharan NaidooShabnum Meer
Jul 29, 2021·Applied Immunohistochemistry & Molecular Morphology : AIMM·Anna Luíza D AraújoOslei P de Almeida

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Methods Mentioned

BETA
X-ray
biopsy
SMA

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