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 withthoracic medullary hemangioblastoma complicated with syringomyelia as the initial presentation

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。

Abstract: 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.