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兄弟姐妹中的多发性栅栏状包膜神经瘤:一例报告及已发表文献综述

Multiple palisaded encapsulated neuromas in siblings: A case report and review of the published work.

作者信息

Liu Guo-Yan, Song Hao, Xu Xiu-Lian

机构信息

Affiliated Hospital of Weifang Medical College, Weifang, China.

Institute of Dermatology, Chinese Academy of Medical Sciences, Nanjing, China.

出版信息

J Dermatol. 2016 May;43(5):560-3. doi: 10.1111/1346-8138.13154. Epub 2015 Oct 13.

Abstract

Palisaded encapsulated neuroma (PEN) is an uncommon, typically solitary, cutaneous neural neoplasm. Multiple mucocutaneous neuromas are usually seen in multiple endocrine neoplasia (MEN) 2b syndrome. Multiple cutaneous PEN in adult patients with or without features of MEN 2b are extremely rare, with only a few cases described. We report a case of multiple PEN in siblings of the same family with no apparent systemic abnormalities, and review the relevant published work to explore its clinical and pathogenic features and the relationship between multiple PEN and MEN 2b type neuroma (multiple mucocutaneous neuromas seen in MEN 2b syndrome).

摘要

栅栏状包膜神经瘤(PEN)是一种罕见的、通常为孤立性的皮肤神经肿瘤。多发性黏膜皮肤神经瘤通常见于多发性内分泌肿瘤(MEN)2b综合征。成年患者中,无论有无MEN 2b特征的多发性皮肤PEN都极为罕见,仅有少数病例报道。我们报告了一例同一家庭中兄弟姐妹患多发性PEN且无明显全身异常的病例,并回顾相关已发表文献,以探讨其临床和致病特征,以及多发性PEN与MEN 2b型神经瘤(MEN 2b综合征中所见的多发性黏膜皮肤神经瘤)之间的关系。

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