Gomes Fernando Cotrim, Larcipretti Anna Laura Lima, Mariano Ítalo Guilherme Giarola De Freitas, Freitas Bruna Carolina Barbosa, Gago Guilherme
Faculty of Medicine, Federal University of Minas Gerais, Belo Horizonte, Brazil.
Hospital das Clínicas, Federal University of Minas Gerais, Belo Horizonte, Brazil.
Neurosurg Rev. 2025 Apr 16;48(1):368. doi: 10.1007/s10143-025-03519-9.
Neurofibromatosis type II (NF2) is a genetic disorder marked by the development of multiple CNS tumors, including meningiomas. Managing NF2-associated meningiomas is complex due to the high tumor burden and the need to preserve neurological function. Gamma Knife radiosurgery (GKRS) offers a minimally invasive approach for targeted tumor control, but data on its long-term safety and efficacy in NF2 patients are limited. This meta-analysis assesses the effectiveness of GKRS for NF2 meningiomas, focusing on tumor control, survival outcomes, and adverse effects. This study was conducted according to the PRISMA guidelines. Pubmed (MEDLINE), Embase, Web of Science, and the Cochrane Library databases were systematically searched for studies reporting clinical outcomes of NFII patients with meningiomas treated with GKRS. Data was analyzed using a random-effects model in R Studio. Six studies, comprising 146 patients and a total of 665 meningiomas, met the inclusion criteria. The median age ranged from 32.5 to 40 years, and the median maximum dose administered ranged from 24.5 to 32 Gy. Local progression at the last follow-up was observed in 41 out of 665 tumors (6%; 95% CI 4-9; I²=45%). Five-year overall survival (OS) was 90% (95% CI 79-96; I²=40%). Radiation-induced adverse effects occurred in 22 patients (18%; 95% CI 8-36; I²=70%), of which 20 were transitory. Mortality due to tumor progression was 17% (95% CI 10-28; I²=48%) at the last follow-up, with no reported cases of malignant transformation among treated lesions. These findings suggest that Gamma Knife radiosurgery provides effective tumor control and favorable survival outcomes in neurofibromatosis II patients with meningiomas, with a relatively low rate of local progression and acceptable safety profile.
II型神经纤维瘤病（NF2）是一种遗传性疾病，其特征是出现包括脑膜瘤在内的多种中枢神经系统肿瘤。由于肿瘤负荷高且需要保留神经功能，管理与NF2相关的脑膜瘤很复杂。伽玛刀放射外科手术（GKRS）为靶向肿瘤控制提供了一种微创方法，但关于其在NF2患者中的长期安全性和有效性的数据有限。这项荟萃分析评估了GKRS治疗NF2脑膜瘤的有效性，重点关注肿瘤控制、生存结果和不良反应。本研究按照PRISMA指南进行。对PubMed（MEDLINE）、Embase、Web of Science和Cochrane图书馆数据库进行系统检索，以查找报告GKRS治疗NFII脑膜瘤患者临床结果的研究。在R Studio中使用随机效应模型分析数据。六项研究，包括146例患者和总共665个脑膜瘤，符合纳入标准。中位年龄在32.5至40岁之间，中位最大剂量在24.5至32 Gy之间。在665个肿瘤中的41个（6%；95%CI 4-9；I²=45%）观察到最后一次随访时的局部进展。五年总生存率（OS）为90%（95%CI 79-96；I²=40%）。22例患者（18%；95%CI 8-36；I²=70%）出现放射诱导的不良反应，其中20例为短暂性。最后一次随访时，肿瘤进展导致的死亡率为17%（95%CI 10-28；I²=48%），治疗的病变中未报告恶性转化病例。这些发现表明，伽玛刀放射外科手术在II型神经纤维瘤病合并脑膜瘤的患者中提供了有效的肿瘤控制和良好的生存结果，局部进展率相对较低，安全性可接受。
