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Morphological features and genetic background in ectomesenchymal chondromyxoid tumor: A systematic review

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dc.contributor.authorJunia de Souza, Raquel Helena
dc.contributor.authorAragão Felix, Fernanda
dc.contributor.authorVasconcelos Filiú, Flávia Martins
dc.contributor.authorPereira de Jesus, Witalo
dc.contributor.authorPaiva Fonseca, Felipe
dc.contributor.authorGomez, Ricardo Santiago
dc.contributor.authorGuimarães Abreu, Lucas
dc.contributor.authorFerreira de Sousa, Sílvia
dc.date.accessioned2025-03-18T08:41:17Z
dc.date.available2025-03-18T08:41:17Z
dc.date.issued2025
dc.description.abstractEctomesenchymal chondro-myxoid tumor (EMCMT) is a rare neoplasm that mainly affects the tongue and harbors recurrent, although not exclusive, gene fusions. Owing to its rarity, overlapping features with other tumors may lead to challenges in the microscopic diagnosis. We aimed to perform a systematic review focusing on the histomolecular findings of EMCMT of the oral and maxillofacial region and to evaluate the possible association between microscopic features with the genetic background. Methods. An electronic search was made on PubMed, Web of Science, Scopus, Ovid, and Embase. Clinicopathological, immunohistochemical, and molecular data were retrieved. Results. Overall, 114 cases from 53 articles on EMCMT were analyzed. Histologically, EMCMT was described as demarcated (84.2%), lobulated (66.7%), reticulated (51.8%), and arranged in sheets, cords, and strands (42.9%), with 73.7% of lesions with spindle-shaped cells. Myxoid stroma (88.6%), chondroid areas (60.5%), chondromyxoid stroma (57.0%), and fibrous septae (42.9%) were also tumor-outlined features. The most expressed markers were vimentin (100.0%), cyclin D1 (100.0%), GFAP (88.5%), NSE (87.5%), S100 (86.5%), CD56 (76.9%), and CD57 (76.5%). The RREB1-MRTFB fusion was detected in 91.0% of the cases investigated and EWSR1 rearrangements in 17.4%. The presence of the fusion RREB1::MRTFB or chromosome alterations in the EWSR1 gene were not highly specific to the morphological features of EMCMT. Conclusion. This study provides a comprehensive summary of the clinicopathological, immunohisto-chemical, and molecular characteristics of EMCMT, aiding in a more accurate microscopic diagnosis of this rare tumor.es
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dc.format.extent11es
dc.identifier.citationHistology and Histopathology Vol. 40, nº04 (2025)
dc.identifier.doihttps://doi.org/10.14670/HH-18-808
dc.identifier.issn0213-3911
dc.identifier.issn1699-5848
dc.identifier.urihttp://hdl.handle.net/10201/151821
dc.languageenges
dc.publisherUniversidad de Murcia, Departamento de Biologia Celular e Histiologiaes
dc.relationSin financiación externa a la Universidades
dc.rightsinfo:eu-repo/semantics/openAccesses
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 Internacional*
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/*
dc.subjectEctomesenchymal chondromyxoid tumores
dc.subjectImmunohistochemistryes
dc.subjectOral neoplasmes
dc.subjectFusion genees
dc.subjectRearrangementes
dc.subject.otherCDU::6 - Ciencias aplicadas::61 - Medicina::616 - Patología. Medicina clínica. Oncologíaes
dc.titleMorphological features and genetic background in ectomesenchymal chondromyxoid tumor: A systematic reviewes
dc.typeinfo:eu-repo/semantics/articlees
dspace.entity.typePublicationes
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Histology and histopathology Vol.40, nº4 (2025)