神经鞘瘤病、神经纤维瘤病2型和幼年性黄色肉芽肿。

Neurilemmomatosis, NF2, and juvenile xanthogranuloma.

作者信息

Iyengar V, Golomb C A, Schachner L

机构信息

Department of Dermatology and Cutaneous Surgery, University of Miami, Florida 33101, USA.

出版信息

J Am Acad Dermatol. 1998 Nov;39(5 Pt 2):831-4. doi: 10.1016/s0190-9622(98)70359-7.

DOI:10.1016/s0190-9622(98)70359-7

PMID:9810909

Abstract

The gene locus for neurilemmomatosis has been reported to lie within the neurofibromatosis type 2 gene region, suggesting that these 2 diseases may be identical. We describe an adolescent girl with multiple neurilemmomas and juvenile xanthogranulomas, who was found to have bilateral acoustic neuromas and multiple central nervous tissue tumors as seen in patients with a diagnosis of neurofibromatosis type 2. This case and recent genetic studies suggest that neurilemmomatosis and neurofibromatosis type 2 are the same disease.

摘要

据报道，神经鞘瘤病的基因位点位于2型神经纤维瘤病基因区域内，这表明这两种疾病可能是相同的。我们描述了一名患有多发性神经鞘瘤和幼年性黄色肉芽肿的青春期女孩，她被发现患有双侧听神经瘤和多个中枢神经组织肿瘤，这与诊断为2型神经纤维瘤病的患者所见相同。该病例及近期的遗传学研究表明，神经鞘瘤病和2型神经纤维瘤病是同一种疾病。