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儿童头皮脑膜上皮错构瘤合并 Gorlin 综合征。

Meningothelial Hamartoma of the Scalp in a Child With Gorlin Syndrome.

机构信息

Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada.

Division of Plastic and Reconstructive Surgery, The Hospital for Sick Children, Toronto, Ontario, Canada ; and.

出版信息

Am J Dermatopathol. 2022 Apr 1;44(4):e39-e40. doi: 10.1097/DAD.0000000000002093.

Abstract

Meningothelial hamartoma of the scalp is a rare entity characterized by a mix of meningothelial tissue and various connective tissue elements. To the best of the authors' knowledge, there has only been one reported case of meningothelial hamartoma of the scalp in the setting of Gorlin syndrome in the literature. In this report, we describe the case of a 3-year-old boy with Gorlin syndrome who presented with a congenital scalp lesion. Histologic examination revealed scattered islands of meningothelial cells in a background of dense fibrous and vascular tissue, in keeping with meningothelial hamartoma of the scalp. The differential diagnoses of congenital scalp lesions and the association between Gorlin syndrome and meningothelial hamartoma of the scalp are discussed.

摘要

头皮脑膜内皮错构瘤是一种罕见的疾病,其特征是脑膜内皮组织和各种结缔组织成分的混合。据作者所知,文献中仅有一例报道的头皮脑膜内皮错构瘤发生在 Gorlin 综合征背景下。在本报告中,我们描述了一例 3 岁男孩患有 Gorlin 综合征,表现为先天性头皮病变。组织学检查显示在密集的纤维和血管组织背景下散布有脑膜内皮细胞岛,符合头皮脑膜内皮错构瘤。讨论了先天性头皮病变的鉴别诊断以及 Gorlin 综合征与头皮脑膜内皮错构瘤之间的关联。

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