摘要
目的回顾分析侵犯中枢神经系统的神经纤维瘤病之临床特点和治疗经验。方法14例诊断明确的神经纤维瘤病患者,Ⅰ型者(2例)主要以神经胶质细胞或神经细胞发育不良(巨脑回)、成瘤性(视神经胶质瘤)以及颅骨缺损为特征;Ⅱ型者(12例)病变主要侵犯脑的被覆组织,如脑膜和神经鞘膜,表现为许旺细胞瘤和脑膜瘤病。针对不同肿瘤类型和主要病征采取个体化治疗方案,其中Ⅰ型蝶骨发育缺损导致进展性突眼者采取眶后壁钛板塑形修补术,脑发育异常引起癫发作者予以药物治疗;Ⅱ型双侧听神经瘤者,根据肿瘤大小、听力损伤程度选择手术切除;有占位效应的并发性肿瘤(多发脑膜瘤、神经鞘瘤)患者也分别采取手术切除;对侧未手术的听神经瘤或多发脑膜瘤、手术后残留听神经瘤复发者施以γ-刀治疗。结果14例患者平均随访27个月,12例Ⅱ型患者中11例经综合治疗后所有肿瘤控制良好,1例死于颅内多发肿瘤侵袭生长。10例13耳行听神经瘤切除术,3例复发,1例死亡;仅3耳保存听力。结论神经纤维瘤病在中枢神经系统中表现多样,多部位、多种类型肿瘤和畸形并存,使治疗十分困难,应当根据不同病变类型和主要的临床症状采取个体化的治疗方案。
Objective To summarize the clinical features and treatment of neurofibromatosis in central nervous system. Methods There were 14 patients confinnly diagnosed as neurofibromatosis （NF）. The main features of patients with NF-type 1 （NF-1, n = 2） were gliocyte or neurocyte dysplasia （macrogyria）, tumorigenesis （optic glioma） and skull defect. NF-type 2 （NF-2, n = 12） was generally involving coating tissue, such as meninges and neurilemma, presenting as schwannoma and meningiomatosis. Individual therapeutic regimens were given according to different types and symptoms of the disease. NF-1 patients with defected sphenoid bone induced progressive exophthalmus received postorbital wall Ti-plate moulding prosthesis, those with encephalodysplasia induced seizure were given drug treatment. NF-2 patients with bilateral acoustic neurinoma, according to tumor size and the extent of hearing disability, were treated by selective resectional therapy, those who complicated with occupancy lesion （such as multi-meningioma, neurinoma） were performed by resection. γ-knife therapy was adopted to the untreated contralateral acoustic neurinoma or multi-meningioma, and postoperative recurrent residual acoustic neurinoma. Results The mean foUow-up period of 14 patients was 27 months. Among 12 NF-2 patients, favourable tumor-control was achieved in 11 cases treated with combination therapy, 1 patient died of intracranial multi-tumor invasion. Thirteen ears in 10 cases were given acoustic neurinoma resection, 3 cases recurred, 1 died, and only 3 ears retained hearing. Conclusion The manifestations of NF in central nervous system are multiplicity, various location, different types and complication with deformities. It is very difficult to diagnose. Individualized therapeutic regimens must be given according to different types and main clinical symptoms.
出处
《中国现代神经疾病杂志》
CAS
2007年第4期357-363,共7页
Chinese Journal of Contemporary Neurology and Neurosurgery
