Abstract：
(1) Background: This study aimed to evaluate the efficacy and treatment-related toxicity of proton radiotherapy (PRT) for vestibular schwannoma (VS) in patients with neurofibromatosis type 2-related schwannomatosis (NF2). (2) Methods: Consecutive NF2 patients treated with PRT for VS between 2004 and 2016 were retrospectively evaluated, focusing on tumor volume, facial and trigeminal nerve function, hearing, tinnitus, vestibular symptoms, and the need for salvage therapy after PRT. (3) Results: Eight patients were included (median age 36 years, 50% female). Median follow-up was 71 months. Five (63%) patients received fractionated PRT and three (38%) received PRT radiosurgery for VS. Six patients (75%) received prior VS surgery; three also received bevacizumab. Six patients (75%) did not require salvage therapy after PRT. Two patients (25%) with residual hearing lost it after PRT, and six had already lost ipsilateral hearing prior to PRT. Tumor and treatment-related morbidity could be evaluated in six patients. Following PRT, conditions that occurred or worsened were: facial paresis in five (83%), trigeminal hypoesthesia in two (33%), tinnitus in two (33%), and vestibular symptoms in four patients (67%). (4) Conclusion: After PRT for VS, the majority of the NF2 patients in the cohort did not require additional therapy. Tumor and/or treatment-related cranial nerve deficits were common. This is at least partly explained by the use of PRT as a salvage treatment after surgery or bevacizumab, in the majority of cases. There remains the further opportunity to elucidate the efficacy and toxicity of proton radiotherapy as a primary treatment.
摘要：
（1）背景：本研究旨在评估质子放疗（PRT）治疗2型神经纤维瘤相关神经鞘瘤病（NF2）患者前庭神经鞘瘤（VS）的疗效和治疗相关毒性。(2)方法:回顾性分析2004年至2016年连续使用PRT治疗的NF2患者,关注肿瘤体积，面部和三叉神经功能，听力,耳鸣,前庭症状,以及PRT后需要抢救治疗。(3)结果:纳入8例患者(中位年龄36岁,50%女性）。中位随访时间为71个月。五名（63％）患者接受了分段式PRT，三名（38％）患者接受了VS的PRT放射外科治疗。六名患者（75％）接受了VS手术；三名还接受了贝伐单抗。6例患者（75％）在PRT后不需要抢救治疗。两名患者（25％）的残余听力在PRT后丧失，在PRT之前，有6人已经失去了同侧听力。可以在6例患者中评估肿瘤和治疗相关的发病率。PRT之后,发生或恶化的病症是：面部轻瘫中有5人(83%)，两例三叉神经感觉减退（33%），二耳鸣（33%），4例患者出现前庭症状（67%）。(4)结论:VSPRT后,队列中大多数NF2患者不需要额外治疗.肿瘤和/或治疗相关的颅神经缺陷很常见。这至少部分解释为使用PRT作为手术后的挽救治疗或贝伐单抗，在大多数情况下。还有进一步的机会来阐明质子放射疗法作为主要治疗的功效和毒性。
