Severe pancreatic involvement in three generations in von Hippel-Lindau disease.

Von Hippel-Lindau disease is a hereditary neoplastic disorder that is most commonly manifested as vascular tumors of the retina and cerebellum. Although visceral involvement is uncommon and is almost always clinically silent, we have encountered three closely related patients with extensive symptomatic pancreatic involvement. One patient had pronounced exocrine pancreatic insufficiency.