以胸髓血管母细胞瘤合并脊髓空洞为首发表现的Von-Hippel Lindau综合征1例报告  

A case report of Von-Hippel Lindau syndrome with thoracic medullary hemangioblastoma complicated with syringomyelia as the initial presentation

作　　者：钱东 赵丹旭 刘春光 章顶立 李宏廷 徐学君 QIAN Dong;ZHAO Danxu;LIU Chunguang;ZHANG Dingli;LI Hongting;XU Xuejun(Graduate School,Zunyi Medical University,Zunyi,Guizhou,563006;Chengdu University of TraditionalChinese Medicine,Chengdu,Sichuan,610075;Department of Neurosurgery,Chengdu City SecondPeople′s Hospital in Sichuan Province,Chengdu,Sichuan,610021)

机构地区：[1]遵义医科大学研究生院,贵州遵义563006 [2]成都中医药大学,四川成都610075 [3]四川省成都市第二人民医院神经外科,四川成都610021

出　　处：《实用临床医药杂志》2022年第2期38-40,共3页Journal of Clinical Medicine in Practice

基　　金：四川省成都市科技局重大科技应用示范项目(2019-YF09-00097-SN)。

摘　　要：Von-Hippel Lindau(VHL)综合征为临床罕见疾病,原发病灶位于胸髓者则更为罕见,目前该病的治疗方法仍以外科手术为主,其中显微镜下行微创手术的治疗效果较好。本研究回顾性分析了1例以胸髓血管母细胞瘤合并脊髓空洞为首发表现的VHL综合征患者的临床资料,以期为该病的诊断与治疗提供参考。Von-Hippel Lindau(VHL) syndrome is a rare clinical disease, and the cases with primary lesion located in the thoracic medulla are rarely seen. At present, surgery is still the main therapy for the VHL syndrome, and the therapeutic effect of microscopic minimally invasive surgery is better. In this paper, we retrospectively analyzed the clinical data of a case of VHL syndrome with thoracic medullary hemangioblastoma complicated with syringomyelia as the initial presentation, in order to provide reference for the diagnosis and treatment of this disease.

关 键 词：Von-Hippel Lindau综合征 胸髓血管母细胞瘤 脊髓空洞 诊断 磁共振成像 

分 类 号：R739.42[医药卫生—肿瘤] R651.2[医药卫生—临床医学]