Von Hippel-Lindau disease

von Hippel-Lindau disease The disease that has perpetuated the names of two prestigious European physicians, Eugen von Hippel and Arvid Lindau, is a familial syndrome characterised by the occurrence of highly vascular tumours in different organs. The main manifestations are haemangioblastomas of the retina and CNS, clear-cell renal cell carcinomas and renal cysts, phaeochromocytomas, neuroendocrine pancreatic tumours and pancreatic cysts, and endolymphatic sac tumours. von Hippel-Lindau disease is an autosomal dominant disease with an incidence of about one in 36 000 people, and with clinical manifestations usually becoming apparent between 18 and 30 years. Clinical Von Hippel-Lindau disease