Some important radiological aspects of the kidney in Hippel-Lindau syndrome: the value of prospective study in an affected family

Abstract

Eighty-three per cent of the affected members of a family with Hippel-Lindau syndrome had hypernephromas and cortical cysts of the kidney. Two of the asymptomatic members proved to have multiple hypernephromas by a prospective study. The carcinomas are frequently multifocal or bilateral. The cortical cysts are usually multiple, bilateral and small, and often contain carcinoma in the wall. The association of multifocal or bilateral hypernephromas with renal cortical cysts represents an important aspect of the Hippel-Lindau syndrome. The value of prospective renal studies in affected families is stressed.