以鞍上血管母细胞瘤为主要表现的VHL综合征手术治疗1例并文献回顾  

Surgical treatment of VHL syndrome with supracellar angioblastoma as the main manifestation:a case report and literature review

作　　者：史涛 林涛 曾永亲 陈志杰 郭东亮 邓斌 李海南 范冲竹 SHI Tao;LIN Tao;ZENG Yongqin;CHEN Zhijie;GUO Dongliang;DENG Bin;LI Hainan;FAN Chongzhu(Department 2 of Neurosurgery,Guangdong 999 Brain Hospital Affiliated to Medical College of Jinan University,Guangzhou 510510,China;不详)

机构地区：[1]广东三九脑科医院神经外二科,广州510510 [2]广东三九脑科医院病理科,广州510510

出　　处：《临床神经外科杂志》2025年第1期117-120,共4页Journal of Clinical Neurosurgery

基　　金：广东省医学科学技术研究基金项目(B2023418)。

摘　　要：目的探讨Von Hippel-Lindau(VHL)综合征的临床特点、影像学表现、病理诊断、治疗方法及预后。方法回顾性分析广东三九脑科医院2021年11月收治的1例以鞍上血管母细胞瘤为主要表现的VHL综合征患者的临床资料,并结合相关文献进行复习。结果患者予右侧翼点入路行鞍上病变切除,术中见病变呈囊实性,推挤同侧视神经致其扁平增宽,分离可见病变血供极其丰富,大部分质韧,予镜下沿边分离断血供后完整切除肿瘤。术后病理检查诊断为血管母细胞瘤。术后随访1年提示鞍上病变未见复发,右侧小脑半球及颈4颈髓病变无明显变化。结论VHL综合征的临床症状因肿瘤的大小和位置而异,主要靠实验室、影像学及基因检测诊断,治疗方法涉及手术及放射治疗,需个体化选择具体治疗方法。Objective To investigate the clinical features,imaging findings,pathological diagnosis,treatment and prognosis of Von Hippel-Lindau(VHL)syndrome.Methods The clinical data of a case of VHL syndrome with supracellar angioblastoma as the main manifestation who were admitted to Guangdong 999 Brain Hospital in November 2021 were reviewed and summarized.Results Supracellar lesion resection was performed through right pterion approach,and intraoperative lesions were cystic and solid,flattened and widened by squeezing the ipsilateral optic nerve.Most of the tumor was tough.The tumor was completely resected after the blood supply was cut off along the edge under the microscope.The postoperative pathological examination diagnosed angioblastoma.The 1-year follow-up showed no recurrence of supramellar lesions,and the lesions in the right cerebellar hemisphere were larger than before.Conclusions The clinical symptoms of VHL syndrome vary with tumor size and location,and the diagnosis mainly depends on laboratory,imaging and genetic testing.Specific treatment methods need to be selected individually.

关 键 词：鞍上血管母细胞瘤 VHL综合征 肾囊肿 脊髓 PVHL 

分 类 号：R743[医药卫生—神经病学与精神病学]