von Hippel-Lindau病影像学诊断

Imaging Diagnosis of von Hippel-Lindau Disease

目的探讨von Hippel-Lindau病(VHL病)的影像学特征。资料与方法回顾性分析7例VHL病影像学表现以及临床、病理资料。结果7例VHL病影像学表现包括均有1处或1处以上的中枢神经系统血管母细胞瘤,5例有1处或多处包括胰腺多发囊肿、双肾多发肿瘤和囊肿以及阴囊肿瘤在内的内脏病变。结论VHL病为家族性遗传性多系统肿瘤综合征,熟悉其影像学表现有利于正确诊断。

Objective To discuss imaging features of yon Hippel-Lindau disease（VHL disease）. Materials and Methods Imaging findings and clinical and pathological data of 7 cases with VHL diseases were retrospectively analyzed. Results Of the 7 eases, imaging findings consisted of CNS hemangioblastomas in one or more than one sites in all eases and visceral lesions in 5 cases. Visceral lesions included pancreatic cysts, renal tumors and cysts and scrota] tumor. Conclusion VHL disease is a heritanee family multi systems neoplasm syndrome. Familiarity with the imaging features of VHL will be helpful to the accurate diagnosis.