Von Hippel-Lindau综合征(VHL)是一种常染色体显性遗传的家族性多系统肿瘤综合征,该病少见,临床表现多样化,涉及多个系统,诊断较为困难。特别是以内脏病变为首发表现时极易误诊、漏诊。本文就其相关内脏病变包括胰腺、肾脏、肾上腺、附睾或子宫阔韧带病变的临床特点及影像学表 Von Hippel-Lindau syndrome (VHL) is an autosomal dominant, familial, multisystem tumor syndrome that is uncommon and has multiple clinical manifestations, multiple systems, and difficult diagnosis. In particular, visceral lesions as the first performance is extremely misdiagnosed, missed diagnosis. This article on its related visceral lesions, including the pancreas, kidney, adrenal, epididymis or uterine broad ligament lesions of the clinical features and imaging table