CAJ | 학술논문

目的探讨家族性同一部位血管母细胞瘤(HB)的诊断和治疗.方法回顾分析两个家系2例小脑HB、3例脑干HB的临床资料和手术疗效,结合文献进行讨论.结果两个家系均确诊为希佩尔-林道病(VHL).5例肿瘤全部切除,随访6-48个月,5例KPS评分均≥90分.结论 HB诊断主要依靠MRI,基因检测对VHL病的诊断有重要价值.显微手术技巧可显著提高本病的疗效;术后控制正常灌注压突破综合征、及时发现和处理相关并发症对本病的预后有重要意义.
목적 탐토가족성동일부위혈관모세포류(HB)적진단화치료.방법 회고분석량개가계2례소뇌HB、3례뇌간HB적림상자료화수술료효,결합문헌진행토론.결과 량개가계균학진위희패이-림도병(VHL).5례종류전부절제,수방6-48개월,5례KPS평분균≥90분.결론 HB진단주요의고MRI,기인검측대VHL병적진단유중요개치.현미수술기교가현저제고본병적료효;술후공제정상관주압돌파종합정、급시발현화처리상관병발증대본병적예후유중요의의.
Objective To study the diagnosis and treatment of familical hemangioblastoma with the same parts of central nervous system.Methods The clinical presentation, surgical treatment and outcomes in two families of two cerebellar hemangioblastoma and three hemangioblastoma of brainstem were analyzed retrospectively and literature review was performed.Results Family A was suspected to be VHL, family B was confirmed to be VHL.Five tumors were removed completely.The KPS of all patients were more than 90 during follow - up period ( from 6 months to 48 months ).Conclusion MRI and DSA may be the most important methods for the diagnosis of HBs.Genetic testing bears great value in the diagnosis of VHL.Microsurgical technique play a key role in the curative effect of hemangioblastoma.NPPB could be controlled in the perioperative period and management of complications is important for prognosis.