颅内恶性神经鞘膜瘤临床诊断及治疗

Clinical diagnosis and therapy of intracranial malignant nerve sheath tumors

目的通过分析颅内恶性神经鞘膜肿瘤这一罕见肿瘤的临床资料,加深对这一疾病的认识,以期提高治疗效果。方法回顾性分析我院1994年1月～2006年11月手术切除并经病理证实的11例颅内恶性神经鞘膜肿瘤患者的临床资料。结果本组病例年龄为3～51岁,男女病例比例为7:4:多数有颅神经障碍,局部肿块以及原有症状再度出现是本组病人的主要临床表现;这些病变起源可分为:(1)直接起源于颅神经根的;(2)非明确神经根起源的;(3)复发并恶变的。肿瘤全切除5例,次全切除3例,部分切除3例。术后近期效果较好,3例辅以放疗。术后3例存活超过5年,存活最长的病例超过10年。结论颅内恶性神经鞘膜肿瘤多见于儿童及中年患者,多数病人病程较短,临床症状因部位不同表现相应神经功能障碍,术后近期效果较好,肿瘤全切除及放疗可提高5年生存率。

Objective To understand more about intracranial malignant nerve sheath tumors （MNST） by analyzing the clinical data of a group of patients with this kind of rare tumor, in order to improve treatment effects. Methods From January 1994 to November 2006, 11 cases of intracranial MNST were diagnosed pathologically and treated at our department. Their clinical data were retrospectively analyzed. Results There were 7 male and 4 female patients ranging in age from 3 to 51 years. Most of them had cranial neurological disorder, and main clinical manifestations included local swell and their previous symptom recurrence. Three subgroups were categorized by their origins： one was from cranial nerve root, another was from unspecified central nerve root, and the other had malignant transformation after recurrence. 5 cases had total removal of tumors, 3 cases subtotal removal, and 3 partial removal. Short-term effects were good. 3 cases underwent radiotherapy. Though these patients recovered quickly after operations, 3 cases survived longer than 5 years, and the longest life span was more than 10 years. Conclusion Such a tumor often occurs in children or middle-aged people. The illness course is short in most patients. Clinical manifestations are mainly neurological disorders of the site where the tumor is located on. The short-term curative effects are satisfied. The complete resection and radiotherapy can promote the 5-year survival rate.