Hippel-Lindau disease. A case history of one family.
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.
D000328:Adult; D000798:Angiomatosis / Q000235:genetics*; D005260:Female; D005817:Genetic Counseling; D006801:Humans; D007680:Kidney Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011859:Radiography; D006623:von Hippel-Lindau Disease / Q000000981:diagnostic imaging / Q000235:genetics* / Q000473:pathology