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病例报告:1例腭部含基因重排的黏液表皮样癌透明细胞变异型儿科病例。

Case Report: A pediatric case of the clear-cell variant of mucoepidermoid carcinoma in the palate harboring gene rearrangement.

作者信息

Wang Hengkun, Wang Xiaoya

机构信息

Department of Stomatology, Weihai Municipal Hospital, Cheeloo College of Medicine, Shandong University, Weihai, China.

出版信息

Front Pediatr. 2025 Jul 3;13:1600823. doi: 10.3389/fped.2025.1600823. eCollection 2025.

DOI:10.3389/fped.2025.1600823
PMID:40677284
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12267255/
Abstract

The clear-cell variant of mucoepidermoid carcinoma (MEC) is a rare subtype, with pediatric cases being extremely rare. A 13-year-old girl presented with a lesion on the right palate, which had been noted for 3 months. The patient reported pain, rupture, and bleeding of the tumor for nearly a month. Computed tomography scans revealed a quasi-circular soft tissue mass on the right hard palate. Microscopically, the tumor cells showed predominant clear cells and scattered mucous cells. The tumor cells were positive for cytokeratin (CK), CK7, CK5/6, epithelial membrane antigen, P63, and P40. A rearrangement of mastermind-like transcriptional coactivator 2 () (11q21) gene was identified in the tumor cells by fluorescence hybridization. The histological features supported a diagnosis of clear-cell variant of MEC, medium grade, with a tumor stage of pT1N0M0. The patient underwent a complete excision of the palatal mass followed by superficial bone removal. After surgery, the patient recovered well and was recurrence-free at the 1-year follow-up. Based on repeated pathological evaluations, we report this rare pediatric case of a clear-cell variant of MEC of the palate. Only surgical resection resulted in a favorable outcome.

摘要

黏液表皮样癌(MEC)的透明细胞变体是一种罕见的亚型,小儿病例极为罕见。一名13岁女孩右侧腭部出现肿物,已被发现3个月。患者自述肿瘤近1个月来出现疼痛、破溃及出血。计算机断层扫描显示右侧硬腭有一近似圆形的软组织肿块。显微镜下,肿瘤细胞以透明细胞为主,散在分布黏液细胞。肿瘤细胞细胞角蛋白(CK)、CK7、CK5/6、上皮膜抗原、P63及P40均呈阳性。通过荧光原位杂交在肿瘤细胞中检测到易位相关转录共激活因子2(MAML2)(11q21)基因重排。组织学特征支持诊断为中等级别的MEC透明细胞变体,肿瘤分期为pT1N0M0。患者接受了腭部肿物完整切除及浅表骨切除。术后患者恢复良好,1年随访无复发。基于多次病理评估,我们报告这例罕见的小儿腭部MEC透明细胞变体病例。仅手术切除就取得了良好疗效。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c20/12267255/9889f2f8febd/fped-13-1600823-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c20/12267255/179f83ffe2a7/fped-13-1600823-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c20/12267255/9889f2f8febd/fped-13-1600823-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c20/12267255/179f83ffe2a7/fped-13-1600823-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/5c20/12267255/9889f2f8febd/fped-13-1600823-g002.jpg

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本文引用的文献

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Clear-cell Variant of Mucoepidermoid Carcinoma Presenting as a Palatal Mass in a 10-Year-old Boy.10 岁男孩腭部肿块:黏液表皮样癌透明细胞变异型。
Anticancer Res. 2023 Feb;43(2):939-942. doi: 10.21873/anticanres.16238.
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