VHL and neoplasm: Von Hippel-Lindau is an autosomal dominant disorder that predisposes individuals to the development of multisystemic neoplasms.24It is caused by pathogenic variants in theVHLgene (OMIM 608537), a tumor suppressor located on the short arm of chromosome 3, which encodes the VHL protein.24The incidence of VHL is ∼1 in 36,000 live births.24