Hippel-Lindau disease. A case history of one family.

Abstract

This article describes the results of a prospective study in the 4 asymptomatic members of a family affected with Hippel-Lindau disease after the death of 2 patients and the surgical findings of the third. It stresses the multicentric and bilateral origin of renal cell carcinoma in Hippel-Lindau disease, usually combined with renal cortical cysts. The walls of these cysts often contain carcinoma cells. It is important that all relatives of a patient with the Hippel-Lindau disease are adequately screened.