NF2相关神经鞘瘤病（NF2-SWN）中前庭神经鞘瘤对氯沙坦的反应：病例系列研究

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中文摘要

目的： 确定NF2相关神经鞘瘤病中前庭神经鞘瘤对单用氯沙坦或联合其他治疗的反应率。
研究设计： 单机构回顾性病例系列研究。
地点： 三级转诊中心。
患者： 20例NF2相关神经鞘瘤病患者。
干预： 单用氯沙坦、氯沙坦联合其他治疗（贝伐珠单抗、布格替尼、阿昔替尼）。
主要结局指标： 体积进展，定义为前庭神经鞘瘤大小变化>20%，依据神经纤维瘤病和神经鞘瘤病反应评估（REiNS）标准。听力学进展定义为听力指标变化，即纯音平均听阈（PTA）增加10 dB或以上，或言语识别率（WRS）下降10%或以上。
结果： 本研究纳入2014年至2023年在本机构接受治疗的20例患者，共33个前庭神经鞘瘤。氯沙坦治疗持续时间范围为8.09至153.04个月（平均：53.8，标准差：37.32）。12个前庭神经鞘瘤同时接受抗肿瘤治疗。在21个单用氯沙坦治疗的前庭神经鞘瘤中，体积缩小、进展和稳定分别见于23.8%、47.6%和28.6%的肿瘤。在5个接受氯沙坦联合贝伐珠单抗治疗的前庭神经鞘瘤中，联合治疗期间40%的肿瘤缩小，20%进展，40%保持稳定。一个在贝伐珠单抗单药治疗期间进展的肿瘤在联合治疗后达到体积稳定。贝伐珠单抗单药治疗期间的体积增长率与联合治疗后的增长率无显著差异。
结论： 本病例系列描述了接受氯沙坦治疗的NF2-SWN患者的反应率。氯沙坦在调节肿瘤大小方面的潜在作用，无论是单用还是与贝伐珠单抗联合，均应通过良好对照的研究进一步研究。本研究受样本量小的限制。

英文摘要

OBJECTIVE: Identify response rates of vestibular schwannomas in NF2-related Schwanomatosis to treatment with losartan alone or combined with other treatments.
STUDY DESIGN: Single-institution retrospective case series.
SETTING: Tertiary referral center.
PATIENTS: Twenty patients with NF2-related Schwannomatosis.
INTERVENTIONS: Losartan alone, Losartan plus other therapy (bevacizumab, brigatinib, axitinib).
MAIN OUTCOME MEASURES: Volumetric progression, defined as a change in VS size >20%, in accordance with the Response Evaluation in Neurofibromatosis and Schwannomatosis (REiNS) criteria. Audiometric progression is defined as a change in hearing measures, either a 10 dB or greater increase in PTA or a 10% or greater decrease in WRS.
RESULTS: This study included 20 patients, with 33 VSs, treated at our institution from 2014 to 2023. Losartan treatment duration ranged from 8.09 to 153.04 months (mean: 53.8, SD: 37.32). Twelve VSs were concurrently managed with antineoplastic therapy. Among 21 VSs treated with losartan alone, volumetric regression, progression, and stability were seen in 23.8%, 47.6%, and 28.6% of tumors, respectively. Among 5 VSs treated with combination losartan and bevacizumab, 40% of tumors regressed, 20% progressed, and 40% remained stable during the combination period. One tumor that progressed during bevacizumab monotherapy treatment achieved volumetric stability following combination therapy. Volumetric growth rates during bevacizumab alone did not significantly differ from growth rates following combination therapy.
CONCLUSIONS: This case series describes response rates among NF2-SWN patients treated with losartan. The potential role of losartan in modulating tumor size, either alone or in combination with bevacizumab, should be further studied through well-controlled studies. The study is limited by a small sample size.