儿童中枢神经系统血管母细胞瘤的显微手术治疗

Hemangioblastomas of the central nervous system in children: microsurgical management

目的探讨儿童中枢神经系统血管母细胞瘤的临床表现、影像学特征、手术治疗及预后。方法回顾性分析四川大学华西医院2000年6月至2012年1月手术及病理证实的儿童中枢神经系统血管母细胞瘤19例。结果本组肿瘤均获全切,术后15例症状好转,4例无明显改善。9例诊断为Von Hippel-Lindau病,2例诊断为散发性血管母细胞瘤,8例未做基因检测未能确定是否为VHL病。结论儿童中枢神经系统血管母细胞瘤平均术前症状持续时间较成人短,以呕吐就诊者较多。术前检查与随访首选全神经轴增强MRI。儿童中枢神经系统血管母细胞瘤以手术治疗为主,若有症状或肿瘤生长迅速应尽快手术。VHL病儿童应密切随访。

Objective This study aims to outline the clinical manifestations,imaging features,surgical treatment and prognosis of children with hemangioblastonas.Methods Pediatric patients with hemangioblastomas （〈 14 years,n =19） treated in Neurosurgical Department of West China Hospital of Sichuan University from June 2000 to January 2012 were reviewed retrospectively.Nineteen children were performed microsurgery for intracranial or spinal hemangioblastomas.All patients of hemangioblastoma were confirmed by pathology.Results Total tumor removal was achieved in all patients.Postoperative condition was improved in 15 patients and no change in 4 patients.In follow-up,the condition of all survibing patients were improved.Two patients were diagnosed as sporadic tumors,nine patients were affected by yon Hippel-Lindau disease （VHL） and eight could not be confirmed without genetic testing and other clinical manifestations of VHL.Conclusion Pediatric hemangioblastoma patients of central nervous system often have a much shorter duration of the preoperative symptons than adults and the vomiting is the most common complain.Contrast-enhance magnetic resonance imaging （MRI） is the most sensitive and accurate imaging modality for discovery and tracking chanfes in hemangioblastomas of central nervous system.Operation is reconmmded to every symptomatic child as early as possible and complete resection is the preferred choice.In asymptomatic children,surgery is recommended if tumor quick growth was observed on MRI in three months.In VHL children,close follow-up is necessary.