多发性细胞性神经鞘瘤——一例报告及免疫组化作为组织学辅助手段作用的综述

Multiple cellular neurothekeomas--a case report and review on the role of immunohistochemistry as a histologic adjunct.

作者信息

Mahalingam M, Alter J N, Bhawan J

机构信息

Quest Diagnostics Incorporated, 415 Massachusetts Avenue, Cambridge, MA, USA.

出版信息

J Cutan Pathol. 2006 Jan;33(1):51-6. doi: 10.1111/j.0303-6987.2006.00400.x.

DOI:10.1111/j.0303-6987.2006.00400.x

PMID:16441413

Abstract

BACKGROUND

Cellular neurothekeoma is a relatively rare, benign cutaneous neoplasm, which usually presents as a solitary papule or nodule involving the head and neck area of young adults. Multiple neurothekeomas have not, to date, been known to occur.

METHODS

We report a 30-year-old, otherwise healthy, male who presented with multiple neurothekeomas (15) in the head and neck area over a period of 12 years.

RESULTS

While the unifying feature of all biopsied (10 of 15) lesions was the presence of epithelioid cells--the lesions differed in their cellularity and the degree of sclerosis of the stromal component. Antigenic profiling of the lesional cells revealed expression of vimentin, NKI/C3, PGP 9.5, factor XIIIa and CD68 but not S100, HMB45, MelanA, EMA, MSA, desmin, CD57 or NGF-R.

CONCLUSIONS

This case report is the first to document the occurrence of multiple cellular neurothekeomas. An unusual histologic feature of some of the biopsied lesions was the presence of a markedly sclerotic stroma.

摘要

背景

细胞性神经鞘黏液瘤是一种相对罕见的良性皮肤肿瘤，通常表现为累及年轻成年人头颈部区域的孤立性丘疹或结节。迄今为止，尚未发现有多发性神经鞘黏液瘤的病例。

方法

我们报告了一名30岁、身体健康的男性，在12年的时间里，其头颈部出现了多发性神经鞘黏液瘤（15个）。

结果

虽然所有活检（15个中的10个）病变的共同特征是存在上皮样细胞，但这些病变在细胞密度和间质成分的硬化程度方面存在差异。对病变细胞进行抗原分析显示，波形蛋白、NKI/C3、PGP 9.5、因子ⅩⅢa和CD68呈阳性表达，但S100、HMB45、MelanA、EMA、MSA、结蛋白、CD57或NGF-R呈阴性表达。