Nguyen Thien, Chung Lawrance K, Sheppard John P, Bhatt Nikhilesh S, Chen Cheng Hao Jacky, Lagman Carlito, Kaprealian Tania, Lee Percy, Nghiemphu Phioanh L, Yang Isaac
Department of Neurosurgery, David Geffen School of Medicine, University of California-Los Angeles, Los Angeles, CA, USA.
Department of Radiation Oncology, David Geffen School of Medicine, University of California-Los Angeles, Los Angeles, CA, USA.
Neurosurg Rev. 2019 Mar;42(1):85-96. doi: 10.1007/s10143-017-0904-2. Epub 2017 Sep 13.
Neurofibromatosis type 2 (NF2) is a genetic neoplastic disorder that presents with hallmark bilateral vestibular schwannomas and multiple meningiomas. Though the current standard of care for meningiomas includes surgery, the multiplicity of meningiomas in NF2 patients renders complete resection of all developing lesions infeasible. Stereotactic radiosurgery (SRS) may be a viable non-invasive therapeutic alternative to surgery. We describe a particularly challenging case in a 39-year-old male with over 120 lesions who underwent more than 30 surgical procedures, and review the literature. We also searched three popular databases and compared outcomes of SRS versus surgery for the treatment of multiple meningiomas in patients with NF2. A total of 50 patients (27 radiosurgical and 23 surgical) were identified. For patients treated with SRS, local tumor control was achieved in 22 patients (81.5%) and distal control was achieved in 14 patients (51.8%). No malignant inductions were observed at an average follow-up duration of 90 months. Complications in the SRS-treated cohort were reported in 9 patients (33%). Eight patients (29.6%) died due to disease progression. Six patients experienced treatment failure and required further management. For NF2 patients treated with surgery, 11 patients (48%) showed tumor recurrence and 10 patients (43.5%) died due to neurological complications. SRS may be a safe and effective alternative for NF2-associated meningiomas. Further studies are required to identify the ideal radiosurgical candidate.
2型神经纤维瘤病（NF2）是一种遗传性肿瘤疾病，其特征为双侧前庭神经鞘瘤和多发性脑膜瘤。尽管目前脑膜瘤的标准治疗方法包括手术，但NF2患者的脑膜瘤数量众多，使得完全切除所有发展中的病变变得不可行。立体定向放射外科（SRS）可能是一种可行的非侵入性手术替代治疗方法。我们描述了一名39岁男性的特别具有挑战性的病例，该患者有超过120个病变，接受了30多次外科手术，并回顾了相关文献。我们还搜索了三个流行的数据库，并比较了SRS与手术治疗NF2患者多发性脑膜瘤的结果。共确定了50例患者（27例接受放射外科治疗，23例接受手术治疗）。对于接受SRS治疗的患者，22例（81.5%）实现了局部肿瘤控制，14例（51.8%）实现了远处控制。在平均90个月的随访期内未观察到恶性诱导。接受SRS治疗的队列中有9例患者（33%）报告了并发症。8例患者（29.6%）因疾病进展死亡。6例患者治疗失败，需要进一步治疗。对于接受手术治疗NF2患者，11例（48%）出现肿瘤复发，10例（43.5%）因神经并发症死亡。SRS可能是NF2相关脑膜瘤的一种安全有效的替代治疗方法。需要进一步研究以确定理想的放射外科治疗候选者。
