Marsan Alexandre Archambault, Chevarie-Davis Myriam, Doyon Josée, Abikhzer Joel, Sirois Jennifer, Berdugo Jérémie
Department of Pathology, University of Montreal, Montreal, QC, Canada.
Department of Oral Surgery, Pierre-Le Gardeur Hospital, Terrebonne, QC, Canada.
Head Neck Pathol. 2025 Jul 14;19(1):83. doi: 10.1007/s12105-025-01806-x.
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.
We report two cases of extra-glossal EMCMTs with RREB1-MRTFB fusions, one being the first from the sinonasal tract to be described.
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.
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。
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。
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。