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Introduction <p>Ectomesenchymal chondromyxoid tumor (EMCMT) is a rare benign tumor of the oral cavity that nearly always occurs in the anterior dorsal aspect of the tongue and has an indolent clinical course. Most EMCMTs harbor a RREB1-MRTFB rearrangement.</p> Materials and methods <p>We report two cases of extra-glossal EMCMTs with RREB1-MRTFB fusions, one being the first from the sinonasal tract to be described.</p> Conclusion <p>In addition to other reported extra-glossal tumors harboring RREB1-MRTFB fusions, our cases expand the spectrum of tumors carrying this rearrangement and raise awareness for extra-glossal locations of EMCMTs.</p>

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Unusual Locations of Extra-Glossal Ectomesenchymal Chondromyxoid Tumors with RREB1–MRTFB Gene Fusions: A Report of Two Cases

  • 期刊论文

  • 发表日期：

    2025年7月14日

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  • Alexandre Archambault Marsan,
  • Myriam Chevarie-Davis,
  • Josée Doyon,
  • Joel Abikhzer,
  • Jennifer Sirois,
  • Jérémie Berdugo

摘要

Introduction

Ectomesenchymal chondromyxoid tumor (EMCMT) is a rare benign tumor of the oral cavity that nearly always occurs in the anterior dorsal aspect of the tongue and has an indolent clinical course. Most EMCMTs harbor a RREB1-MRTFB rearrangement.

Materials and methods

We report two cases of extra-glossal EMCMTs with RREB1-MRTFB fusions, one being the first from the sinonasal tract to be described.

Conclusion

In addition to other reported extra-glossal tumors harboring RREB1-MRTFB fusions, our cases expand the spectrum of tumors carrying this rearrangement and raise awareness for extra-glossal locations of EMCMTs.