神经纤维瘤病 2 型（NF2）是一种常染色体显性遗传疾病，其特征为双侧前庭神经鞘瘤（VS）、脑膜瘤、室管膜瘤、脊柱和周围神经鞘瘤、视神经胶质瘤和青少年白内障。正在进行的研究为 NF2 基因和梅林在 VS 肿瘤发生中的作用提供了新的见解。随着对 NF2 肿瘤生物学的了解日益加深，针对特定分子途径的治疗已经开发并在临床前和临床研究中进行了评估。NF2 相关VS是重要的致病因素，目前的治疗方法包括手术、放射治疗和观察。目前，没有经FDA批准的针对VS的医疗治疗方法，有选择性的治疗方法的开发是一个高优先级的问题。本文综述了 NF2 肿瘤生物学以及目前正在研究用于治疗 VS 患者的现有治疗方法。© 2023年，作者（S）独家授权Springer Science+Business Media，LLC，隶属于Springer Nature。

Neurofibromatosis 2 (NF2) is an autosomal-dominant genetic disorder characterized by bilateral vestibular schwannomas (VS), meningiomas, ependymomas, spinal and peripheral schwannomas, optic gliomas, and juvenile cataracts. Ongoing studies provide new insight into the role of the NF2 gene and merlin in VS tumorigenesis.As NF2 tumor biology becomes increasingly understood, therapeutics targeting specific molecular pathways have been developed and evaluated in preclinical and clinical studies. NF2-associated VS are a source of significant morbidity with current treatments including surgery, radiation, and observation. Currently, there are no FDA-approved medical therapies for VS, and the development of selective therapeutics is a high priority. This manuscript reviews NF2 tumor biology and current therapeutics undergoing investigation for treatment of patients with VS.© 2023. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.