【摘 要】目的：探讨颅内原发恶性神经鞘瘤的临床特点、治疗方法及预后。方法：对1例经免疫组化诊断为原发恶性前庭神经鞘瘤进行资料收集，对中枢神经系统恶性神经鞘瘤的文献进行分析。结果：经检查发现肿瘤后，经显微镜下行肿瘤次全切除术，免疫组化结果为“低度恶性神经鞘瘤”。术后经伽玛刀辅助放疗，随访3个月，病灶未见复发。结论：颅内恶性神经鞘瘤发病率低，治疗上首先考虑手术切除，术后辅助放疗可降低局部复发率，但长期预后较差。

    【关键词】颅内原发恶性神经鞘瘤；临床特点；治疗；预后

    Abstract Objective： To investigate the clinical characteristics， treatment and prognosis of primary intracranial malignant schwannoma.Methods：1 cases with immunohistochemical diagnosis of primary malignant schwannoma were collected， analysis of central nervous system malignant schwannoma literature. The results of the inspection：found after tumor after subtotal resection of tumors under microscope ......