2型神经纤维瘤病合并颅内脑膜瘤的临床特点和手术疗效

Clinical features and surgical treatment of meningiomas in neurofibromatosis type 2 patients

目的探讨2型神经纤维瘤病（NF2）合并颅内脑膜瘤的临床特点与手术治疗。方法回顾性分析2001年1月至2015年7月首都医科大学附属北京天坛医院神经外科收治的18例NF2合并颅内脑膜瘤患者的临床资料。所有患者均采用显微外科手术切除肿瘤，术后对所有患者行临床及影像学随访。结果18例患者共76个脑膜瘤，手术切除21个，其中19个为全切除（SimpsonⅠ或Ⅱ级），2个为大部分切除（SimpsonⅣ级）；9个位于幕上（6个位于大脑镰旁，3个位于大脑凸面），直径为4．0～8．7cm，平均（5．7±1．9）cm；11个位于颅底（6个位于枕大孔区、4个位于中颅窝底、1个位于小脑幕），直径为1．5—3．7cm，平均（2．7±0．6）cm；1个位于第四脑室内。18例患者中，11例术后病情平稳，5例出现术后感染，2例出现新发神经功能缺损症状。出院时14例患者症状较术前改善。病理学检查结果为：5个为移行型或混合型，4个为纤维型，2个为砂砾型，2个为过渡型，1个为内皮型，1个为血管瘤型，1个为非典型性，1个为脑膜瘤和室管膜瘤混杂，4个病理类型不明。18例患者的随访时间为11～97个月，中位随访时间为42．5个月。随访期间，15例患者症状改善，3例无明显变化，未发现手术切除肿瘤复发。结论NF2脑膜瘤以多发为主，可见于颅内不同区域，手术的主要目的是切除肿瘤以改善患者症状并提高患者的生命质量。外科治疗是改善NF2患者预后的重要手段。

Objective To investigate the clinical manifestations and surgical treatment of meningiomas in neurofibromatosis type 2 （NF2） patients. Methods The clinical data of 18 NF2 patients with meningiomas who underwent meningioma resection at Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University from January 2001 to July 2015 were analyzed retrospectively in this study. Long-term follow-up was performed for those patients. Results A total of 18 patients harbored 76 meningiomas. Twenty-one meningiomas in 18 patients were resected. Gross total resection （Simpson grade Ⅰ / Ⅱ ） was performed in 19 tumors and partial removal （ Simpson grade Ⅳ ） in 2. Nine tumors were located at the convexity and cerebral falx and the mean diameter was 5.7 ± 1.9 cm （4.0 - 8.7 cm）. Eleven tumors were located at the skull base including foramen magnum （6/11）, middle cranial fossa （4/11） and tentorium cerebelli （ 1/11 ）. The average diameter was 2.7 ± 0. 6 cm （ 1.5 - 3.7 cm）. One tumor was located in the fourth ventricle. The postoperative course of 11 patients was uneventful. Five patients had postoperative infection. The symptoms and neurologic deficits remained in 2 patients. Symptoms were improved in 14 patients at discharge. The results of pathological examination were ： 5 for mixed type, 4 for fibrous type, 2 for gravel type, 2 for transitional type, 1 for endothelial type, 1 for hemangioma type, 1 for atypical, 1 for mixture of meningioma and ependymoma, and the other 4 pathological types were unknown. The patients were followed up 11 -97 months（ median ：42.5 months）. The symptoms were alleviated in 15 （83%） patients and stable in 3 （ 17% ）. No tumor recurrence or development was observed. Conclusions The patients with NF2 seem to be prone to develop multiple meningiomas which can be located at different regions. The main purpose of surgery is to remove the tumor to improve the patients symptoms and improve the patient＇s quality of life. Surgical treatment plays an important role in improving the prognosis of patients.