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目的 分析中枢神经系统多发性神经鞘瘤病的临床特点及治疗.方法 从2008年1月至2015年6月于首都医科大学附属北京天坛医院神经外科诊治的1 045例椎管鞘瘤患者中,筛选出70例(6.7％)中枢神经系统多发性神经鞘瘤病患者,均行手术治疗,回顾性分析其临床特点及治疗结果.结果 70例中,男/女=49/21(P=0.001),初次行椎管手术的年龄为10 ～72(43.0±14.9)岁,8例(11.4％)有家族史.18例椎管鞘瘤位于颈椎,6例位于上胸段(T1～6),39例位于下胸段(T7～12),58例位于腰椎.30例发生椎管马尾多发小结节,5例合并颅内单发非听神经鞘瘤,24例(34.3％)合并外周神经鞘瘤.57例(81.4％)接受单次椎管手术;13例(18.6％)因新发肿瘤行多次椎管手术,均为非节段性多发性神经鞘瘤病,且初次椎管手术年龄低于单次手术者[分别为(32.9±14.7)、(45.4±14.0)岁;P=0.006].手术全切除率为92.9％ (65/70).术后随访1个月至7年(中位数为50个月),其中痊愈38例(54.3％),好转24例(34.3％),稳定6例(8.6％),加重2例(2.9％).结论 多发性神经鞘瘤病椎管肿瘤多发生于下胸段和腰椎管内,颅内肿瘤少见.节段性患者或初次椎管手术年龄较大者预后相对较好,部分患者可因新发肿瘤而需要多次椎管手术治疗.

Objective To analyze the clinical features and treatment of schwannomatosis in central nervous system.Methods From January 2008 to June 2015, 70 patients (6.7％) with schwannomatosis were screened from 1045 patients with spinal schwannoma treated at the Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University.They were all treated with surgery.Their clinical features and treatment results were analyzed retrospectively.Results Of the 70 patients, 49 were males and 21 were females (P=0.001).The age of first performing spinal operation ranged from 10 to72 (43.0 ±4.9) years.Eight patients (11.4％) had a family history.The spinal schwannomas of 18 patients located in cervical vertebra;6 in the superior thoracic segment (T1-6);39 in the inferior thoracic segment (T7-12);and 58 in the lumbar vertebra.Thirty patients had multiple small cauda equine nodules in spinal canals, 5 complicated with solitary intracranial non-vestibular schwannoma, and 24 (34.3％) complicated with peripheral schwannoma.Fifty-seven patients (81.4％) received a single spinal surgery;13 (18.6％) underwent multiple spinal canal surgery because of newly developed tumors.They were all non-segmental schwannomatosis.The age of the patients with the first spinal canal surgery was younger than the patients with single surgery (32.9 ± 14.7 years vs.45.4 ± 14.0 years;P =0.006).The total resection rate was 92.9％ (65/70).The postoperative follow-up time ranged from 1 month to 7 years (median, 50 months).Thirty-eight (54.3％) of them fully recovered, 24 (34.3％) improved, 6 (8.6％) were stable, and 2 (2.9％) aggravated.Conclusions Spinal canal tumors of schwannomatosis mostly occur in the inferior thoracic segments and within the lumbar tubes.Intracranial tumors are rare.The prognosis of segmental patients or older patients with the first operation is relatively better;part of the patients may need several operations because of the occurrence of new tumors.