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伴有RREB1-MRTFB基因融合的舌外间叶性软骨黏液样瘤的不寻常部位:两例报告

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

作者信息

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

机构信息

Department of Pathology, University of Montreal, Montreal, QC, Canada.

Department of Oral Surgery, Pierre-Le Gardeur Hospital, Terrebonne, QC, Canada.

出版信息

Head Neck Pathol. 2025 Jul 14;19(1):83. doi: 10.1007/s12105-025-01806-x.

Abstract

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.

摘要

引言

外胚间叶性软骨黏液样肿瘤(EMCMT)是一种罕见的口腔良性肿瘤,几乎总是发生于舌背前部,临床病程进展缓慢。大多数EMCMT存在RREB1-MRTFB重排。

材料与方法

我们报告了2例伴有RREB1-MRTFB融合的舌外EMCMT病例,其中1例是首例报道的鼻窦EMCMT。

结论

除其他已报道的伴有RREB1-MRTFB融合的舌外肿瘤外,我们的病例扩大了携带这种重排的肿瘤谱,并提高了对EMCMT舌外发病部位的认识。

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