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Ectomesenchymal Chondromyxoid Tumor of the Oral Cavity: A Report of 5 New Cases with Comprehensive Review of the Literature and Clinicohistopathological Features

Published:December 20, 2022DOI:https://doi.org/10.1016/j.oooo.2022.12.004

      Statement of Clinical Relevance

      • ECT is an uncommon tumor with a wide spectrum of histopathologic and immunohistochemical features. A thorough review of its characteristics is beneficial to the treating clinicians and pathologists as they diagnose and manage patients with this rare neoplasm.

      Abstract

      Objective

      : Ectomesenchymal chondromyxoid tumor (ECT) is an uncommon soft tissue tumor with an enigmatic histogenesis and striking predilection for the tongue. We present 5 new cases and review the literature.

      Study Design

      : We performed a retrospective search for ECTs within the University of Kentucky Oral Pathology Biopsy Service and the published literature.

      Results

      : Five new cases from the biopsy archives and 103 well-documented ECT cases from the literature were compiled and reviewed. 89.8% of ECT are found on the anterior/dorsal/lateral/unspecified tongue, while 4.6% are on the posterior/base of tongue. Six extralingual cases are reported. The age ranges from 2.3-78 years with an average of 40. Most ECT react with GFAP (92.8%) and S-100 protein (91.3%). 21/23 cases demonstrate a RREB1::MKL2 fusion, while EWSR1 gene mutations are identified in 4 cases.

      Conclusions

      : Most ECT are readily diagnosed on routine histopathology in combination with tumor site, immunohistochemical findings, and molecular findings; however, a subset share overlapping features with myoepithelioma of soft parts. As further molecular analysis is performed on this tumor, we may find that a subset of previously diagnosed ECT relate to or represent myoepithelioma or conversely fall under the spectrum of the pluripotent ECT.

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