【摘要】 目的　评价冯·希佩尔·林道病的影像学表现。方法　二个家庭的四个病人接受了头部MR检查 ,腹部超声(US)和 /或CT检查。结果　四个病例呈现了冯·希佩尔·林道病的几乎所有主要临床征象 ,包括视网膜血管瘤、中枢神经系统血管母细胞瘤、内淋巴囊瘤、胰腺囊肿、肾上腺嗜铬细胞瘤、肾囊肿和肾透明细胞癌及附睾乳头状囊腺瘤等。结论　影像学检查在冯·希佩尔·林道病的诊治中仍起着极其重要的作用。更多还原

【Abstract】 Objective To evaluate the imaging manifestation of von Hippel Lindau (VHL) disease. Methods Four patients from two families with von Hippel Lindau disease underwent cranial magnetic resonance imaging (MRI), abdominal ultrasonography (US), and/or CT scans. Results The clinical and imaging manifestations of these cases demonstrated almost all of the major lesions with VHL disease, including retinal angiomas, hemangioblastomas (HBLs) of the central nervous system (CNS), endolymphatic sac tumors (ELSTs), pancreatic cysts, adrenal pheochromocytomas, renal cysts and renal clear cell carcinomas and epididymal cystadenomas. Conclusion Imaging techniques still play an important role in the evaluation and management of VHL disease.更多还原