renal carcinomas and pheochromocytomas. CNS hemangioblastomas are usually surgically removed if they are symptomatic. Photocoagulation and cryotherapy are usually used for the treatment of symptomatic retinal angiomas, although anti-angiogenic treatments may also be an option. Renal tumours may be removed by a partial nephrectomy or other techniques such as radiofrequency ablation. Belzutifan is a drug under investigation for the treatment of von Hippel–Lindau disease-associated renal cell carcinoma. Epidemiology VHL disease has an incidence of one in 36,000 births. There is over 90% penetrance by the age of 65. Age at diagnosis varies from infancy to age 60–70 years, with an average patient age at clinical diagnosis of 26 years. History The German ophthalmologist Eugen von Hippel first described angiomas in the eye in 1904. Arvid Lindau described the angiomas of the cerebellum and spine in 1927. The term Von Hippel–Lindau disease was first used in 1936; however, its use became common only in the 1970s. Notable cases Some descendants of the McCoy family (involved in the Hatfield-McCoy feud of Appalachia, USA) are presumed to have VHL. In an article appearing in the Associated Press, it has been speculated by a Vanderbilt University endocrinologist that the hostility underlying the Hatfield–McCoy feud may have been partly due to the consequences of Von Hippel–Lindau disease. The article suggests that the McCoy family was predisposed to bad tempers because many of them had a pheochromocytoma which produced excess adrenaline and a tendency toward explosive tempers. Nomenclature Other uncommon names are: angiomatosis retinae, familial cerebello-retinal angiomatosis, cerebelloretinal hemangioblastomatosis, Hippel Disease, Hippel–Lindau syndrome, HLS, VHL, Lindau disease or retinocerebellar angiomatosis. See also Haemangioblastoma epithelial sodium channel Nephron proximal convoluted tubule References External links GeneReviews/NCBI/NIH/UW entry on Von Hippel-Lindau Syndrome (VHL gene) Autosomal dominant disorders Rare diseases Hereditary cancers Genodermatoses Syndromes