CT diagnosis of unsuspected von Hippel-Lindau disease

Von Hippel-Lindau disease is a hereditary disorder with complex, multi-organ involvement including retinal, central nervous system, and abdominal manifestations. We report a case of clinically unsuspected von Hippel-Lindau disease identified during computed tomography (CT) evaluation of a renal mass. The CT demonstration of a coexisting pancreatic tumor and renal cell carcinoma suggested the correct diagnosis of von Hippel-Lindau disease, which was subsequently confirmed. This case reemphasizes the value of preoperative assessment of renal tumors by CT. The finding of coexisting renal and pancreatic tumors should stimulate the search for further evidence of von Hippel-Lindau disease.