神经纤维瘤2型患者听神经瘤的立体定向放射外科治疗：一个涉及多个国际中心的反应和恶性转化风险病例系列。
Stereotactic Radiosurgery for Vestibular Schwannoma in Neurofibromatosis Type 2: An International Multicenter Case Series of Response and Malignant Transformation Risk.
发表日期：2023 Mar 02
作者：
Othman Bin-Alamer, Andrew Faramand, Norah A Alarifi, Zhishuo Wei, Arka N Mallela, Victor M Lu, Ahmed M Nabeel, Wael A Reda, Sameh R Tawadros, Khaled Abdelkarim, Amr M N El-Shehaby, Reem M Emad, Selcuk Peker, Yavuz Samanci, Cheng-Chia Lee, Huai-Che Yang, Violaine Delabar, David Mathieu, Manjul Tripathi, Kathryn Nicole Kearns, Adomas Bunevicius, Jason P Sheehan, Tomas Chytka, Roman Liscak, Nuria Martínez Moreno, Roberto Martínez Álvarez, Inga S Grills, Jacob S Parzen, Christopher P Cifarelli, Azeem A Rehman, Herwin Speckter, Ajay Niranjan, L Dade Lunsford, Hussam Abou-Al-Shaar
来源：
NEUROSURGERY
摘要：
神经纤维瘤病2型相关的前庭神经鞘瘤（VSs）是具有挑战性的肿瘤。立体定向放射外科（SRS）的使用不断增加，需要进一步研究它的作用和安全性。为了评估经过SRS治疗后的NF2患者的肿瘤控制、免于额外治疗（FFAT）、实用听力保留和辐射相关风险，我们进行了一项回顾性研究，共纳入了267名NF2患者（328个VSs），这些患者在参加国际放射外科研究基金会的12个中心接受了单次SRS治疗。患者的年龄中位数为31岁（IQR 21-45岁），其中52%为男性。328个肿瘤在中位随访时间为59个月（IQR 23-112个月）期间接受了SRS治疗。在10年和15年时，肿瘤控制率分别为77%（95% CI：69%-84%）和52%（95% CI：40%-64%），FFAT率分别为85%（95% CI：79%-90%）和75%（95% CI：65%-86%）。在5年和10年时，实用听力保留率分别为64%（95% CI：55%-75%）和35%（95% CI：25%-54%）。在多元分析中，年龄（危险比：1.03 [95% CI：1.01-1.05]; P = .02）和双侧VSs（危险比：4.56 [95% CI：1.05-19.78]; P = .04）是实用听力丧失的预测因素。在这个队列中，没有遇到放射诱导性肿瘤或恶性转化的情况。尽管15年时的绝对体积肿瘤进展率为48%，但与VS相关的FFAT率在SRS后的15年达到了75%。在SRS后，没有一位NF2相关的VS患者发生新的辐射相关肿瘤或恶性转化。版权所有©2023作者。 Wolters Kluwer Health, Inc.代表神经外科医师学会(Congress of Neurological Surgeons)出版。
Vestibular schwannomas (VSs) related to neurofibromatosis type 2 (NF2) are challenging tumors. The increasing use of stereotactic radiosurgery (SRS) necessitates further investigations of its role and safety.To evaluate tumor control, freedom from additional treatment (FFAT), serviceable hearing preservation, and radiation-related risks of patients with NF2 after SRS for VS.We performed a retrospective study of 267 patients with NF2 (328 VSs) who underwent single-session SRS at 12 centers participating in the International Radiosurgery Research Foundation. The median patient age was 31 years (IQR, 21-45 years), and 52% were male.A total of 328 tumors underwent SRS during a median follow-up time of 59 months (IQR, 23-112 months). At 10 and 15 years, the tumor control rates were 77% (95% CI: 69%-84%) and 52% (95% CI: 40%-64%), respectively, and the FFAT rate were 85% (95% CI: 79%-90%) and 75% (95% CI: 65%-86%), respectively. At 5 and 10 years, the serviceable hearing preservation rates were 64% (95% CI: 55%-75%) and 35% (95% CI: 25%-54%), respectively. In the multivariate analysis, age (hazards ratio: 1.03 [95% CI: 1.01-1.05]; P = .02) and bilateral VSs (hazards ratio: 4.56 [95% CI: 1.05-19.78]; P = .04) were predictors for serviceable hearing loss. Neither radiation-induced tumors nor malignant transformation were encountered in this cohort.Although the absolute volumetric tumor progression rate was 48% at 15 years, the rate of FFAT related to VS was 75% at 15 years after SRS. None of the patients with NF2-related VS developed a new radiation-related neoplasm or malignant transformation after SRS.Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Congress of Neurological Surgeons.