摘要
目的 探讨VHL病相关性和散发性中枢神经系统(CNS)血管母细胞瘤(HB)临床、处理及预后特征。方法 回顾性分析连续20年中我科收治的66例CNS-HB病人资料。对近期病例作了VHL基因突变和VEGF表达检测。结果 66例HB病人中,散发性58例,VHL相关性8例,共79处病变,7例为多发病变。全组62例共施行了70次手术治疗,其中5例进行了多次手术。8例VHL相关病人中有3例多次手术。64次全切,6次大部分切除。大部分切除的病人有4例复发。手术死亡3人。70次手术中手术效果良好者46次,病情改善者18次,稳定者3次。57例平均随访6.7年(1～16年),41例恢复工作,5例生活自理,部分自理和完全不能自理各1例,9例死亡。VEGF基因突变率为75％(18/24),HB中均见VEGF过度表达。结论 CNS-HB病人的手术治疗结果良好,VHL相关病人的远期愈后较差。
Objective To explore the clinical manifestations, management and prognosis in the patients with central nervous system (CNS) hemangioblastomas (HB) in von Hippel -lindau syndrome (VHL) and sporadic disease. Methods The data of 66 petients with CNS HB treated in our department were analysed retrospectively. VHL gene mutation and VEGF expression were detected in the patients with CNS HB who were recently treated. Results Of 66 patients with HB, 58 were sporadic and 8 related with VHL. The 79 lesions were observed because there were multiple lesions in 8 patients. Seventy operations were performed in all patients, including 5 patients accepted operations more than one time. Of 70 operations, 64 came to total resection of HB and 6 to part. Of 70 operations, 46 were very good operative outcomes, 18 relatively good and 3 produced no changes in the patients. Three patients died after the operation. Of 57 patients followed up for 0.5-16 years (averagely 6.7 years), 41 returned to work, 7 completly or partly took care of themselves and 9 died. The rate of gene mutation is 75% (187 24); High expression of VEGF were observed in HB. Conclusions The operative treatment outcomes of CNS HB patients are good. However, the management of the patients with HB related to VHL is much more difficult than that of ones with sporadic HB. Differentiating the patients with HB related to VHL from the ones with sporadic HB is necessary for correctly predicting the prognosis. Related molecalar genetics determinetion should be performed if it is available.
出处
《中国临床神经外科杂志》
2002年第2期69-71,共3页
Chinese Journal of Clinical Neurosurgery
