CT screening of the abdomen in von Hippel-Lindau disease

Patients with von Hippel-Lindau disease often develop asymptomatic abdominal manifestations including renal cysts and carcinomas, pheochromocytomas, and pancreatic tumors and cysts. Early detection of renal carcinoma and pheochromocytoma is particularly important. Accordingly, periodic abdominal screening of affected individuals and at-risk family members is necessary. Abdominal computed tomography (CT) was performed in 31 subjects from three families. Fifteen had asymptomatic abdominal manifestations of von Hippel-Lindau disease including renal cysts in 13, renal carcinoma in four, pancreatic cysts in five, and pheochromocytomas in two. CT constitutes an excellent noninvasive screening technique for patients with possible abdominal involvement by von Hippel-Lindau disease.