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神经纤维瘤病 2 型相关前庭神经鞘瘤的治疗。

Management of Neurofibromatosis Type 2-Associated Vestibular Schwannomas.

机构信息

Division of Otolaryngology-Head and Neck Surgery, University of California San Diego School of Medicine, 200 West Arbor Drive, MC 8895, San Diego, CA 92103, USA.

Department of Neurosurgery, University of California San Diego School of Medicine, 9300 Campus Point Drive, Mail Code 7893, La Jolla, CA 92037, USA.

出版信息

Otolaryngol Clin North Am. 2023 Jun;56(3):533-541. doi: 10.1016/j.otc.2023.02.012. Epub 2023 Mar 22.

Abstract

Neurofibromatosis type 2 (NF2) is an autosomal dominant syndrome caused by a mutation in the NF2 suppressor gene and is characterized by the development of multiple benign tumors throughout the central nervous system. Bilateral vestibular schwannomas (VSs) are pathognomonic for NF2 and are associated with progressive hearing loss and eventual deafness in most patients. This review presents current management options for NF-2-associated VSs.

摘要

神经纤维瘤病 2 型(NF2)是一种常染色体显性遗传综合征,由 NF2 抑制基因的突变引起,其特征是中枢神经系统内多处良性肿瘤的发展。双侧前庭神经鞘瘤(VSs)是 NF2 的特征性表现,并且与大多数患者的进行性听力损失和最终失聪有关。本文综述了 NF2 相关 VSs 的当前治疗选择。

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