Von Hippel-Lindau disease epidemiology
Incidence: 1 in 31,000 to 36,000 live births. ≈ 30% of patients with cerebellar hemangioblastoma have VHL 1).
Von Hippel-Lindau 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 average patient age at a clinical diagnosis of 26 years.
1)
Hottinger AF, Khakoo Y. Neurooncology of familial cancer syndromes. J Child Neurol. 2009; 24:1526–1535