摘要
目的探讨Von Hippel-lindau(VHL)病的CT及MRI表现。方法回顾性分析8例临床符合VHL病诊断标准患者的CT和MRI表现以及临床病理资料。8例均行中枢神经系统MRI检查,1例行腹部MRI检查,6例行腹部CT检查。结果 8例均有一处或多处中枢神经系统血管母细胞瘤(2例单发于小脑,6例小脑、延髓及脊髓同时发现病变,1例合并幕上病变)。主要表现为囊实性或实性病灶,增强扫描囊实性病灶呈典型的大囊小结节且结节显著强化,实性病灶增强扫描肿瘤结节明显均匀强化,边界清晰。7例有一处或多处内脏病变,主要表现为胰腺多发囊肿、双肾多发肿瘤和囊肿。结论 VHL病CT及MRI表现具有一定特征性,当出现中枢神经系统多发血管母细胞瘤,胰腺多发囊肿、双肾脏多发肿瘤和囊肿时,则高度提示VHL病的可能性。
Objective To analyze CT and MRI characteristics of Von Hippel Lindau（VHL） disease. Methods Ima- ging findings and clinical pathological data of 8 cases with VHL diseases were retrospectively reviewed. All cases underwent CNS MRI scanning, 1 of them underwent abdominal MRI and 6 of them also underwent abdominal CT. Results CNS he- mangioblastomas were found located at cerebellum in 2, involved both spinal marrow and cerebellum in 6, and supratentorial in 1. The lesions presented solid cystic or simple solid tumors. The former showed typical cystic cavity with mural nodules, while the latter showed homogeneous enhancement with clear boundary. There were 1 or more sites of visceral lesions in 7 cases, included pancreatic and renal tumors and cysts. Conclusion VHL disease has certain CT and MRI characteris- tics. Combination of CNS hemangioblastomas, pancreatic cysts, renal tumors and cysts is highly suggestive of VHL dis- ease.
出处
《临床放射学杂志》
CSCD
北大核心
2013年第10期1407-1411,共5页
Journal of Clinical Radiology
