26例中枢神经系统恶性周围神经鞘瘤的临床分析  

Clinical analysis of 26 central nervous system malignant peripheral nerve sheath tumors

作　　者：胡孟庆 赵锦程[2] 任晓辉[1] 赵萌[1] 王军梅[3] 姜中利[1] 

机构地区：[1]首都医科大学附属北京天坛医院神经外科,100050 [2]河北省秦皇岛市第一医院神经外科 [3]北京市神经外科研究所神经病理室

出　　处：《中华神经外科杂志》2014年第5期445-449,共5页Chinese Journal of Neurosurgery

摘　　要：目的探讨中枢神经系统恶性周围神经鞘瘤（MPNST）的诊断、治疗及预后。方法回顾性总结2004年至2011年收治的26例经病理证实的颅内和椎管内MPNST，应用Log-rank法和Cox回归进行统计分析，并结合文献对本病的特点、治疗方法和预后进行分析。结果26例中男16例，女10例。肿瘤位于颅内17例，椎管内9例。临床表现主要有疼痛、神经受累症状等。全切除14例，部分切除12例。共随访到21例，术后随访1．5～102．0个月，其中5例因肿瘤复发而死亡，7例肿瘤复发，9例无明显复发。单因素分析显示：女性、肿瘤全切、原发病变与较好预后相关（P〈0．05）。多因素分析显示：全切除为预后好的独立因素（P=0．006）。结论中枢神经系统MPNST临床罕见，全切除是改善预后的重要的手段，对于未全切除的病例，术后放疗值得推荐。Objective To explore the diagnosis, comprehensive treatment strategies and prognosis for intracranial and intraspinal malignant peripheral nerve sheath tumors （MPNST） . Methods The investigators summarized 26 cases with MPNST which were performed by post-operation pathology from 2004 to 2011. By Log-rank and Cox model analysis, we analyzed the clinical features, radiological manifestations, pathological features, comprehensive treatment strategies and prognosis, and relevant literatures. Results Among the 26 cases,16 cases were male,10 cases were female,17 tumors were in cranial ,9 were located in intraspinal, symptoms mainly included pain and impaired dysfunction of nerves. The follow-up data of 21 patients were collected from 1.5 to 102. 0 months after surgery. Among these patients ,5 patients had died due to tumor recurrence, 7 patients were alive with tumor recurrence and 9 patients had no recurrence. Univariate analysis revealed that female gender, gross total resection, and primary MPNST were significantly associated with favorable prognosis （ P 〈 0. 05 ）. Multivariate analysis revealed only gross total resection to be an independent favorable prognostic factor （ P = 0. 006 ）. Conclusions Intracranial and intraspinal MPNST is a kind of rare neoplasm. The recurrence rate and mortality is higher. Gross total resection whenever possible is necessary for prolonged survivals. For patients with incomplete tumor resection, postoperative radiation therapy is recommended.

关 键 词：恶性周围神经鞘瘤 中枢神经系统 诊断 综合治疗 预后 

分 类 号：R739.43[医药卫生—肿瘤]