von Hippel-Lindau disease (VHL) is an autosomal-dominant neoplasia syndrome that is the
result of a germline mutation of the VHL tumor suppressor gene on the short arm of
chromosome 3. Patients with VHL are predisposed to develop lesions of the central nervous
system and viscera. Central nervous system lesions include hemangioblastomas (the most
common tumor in VHL) and endolymphatic sac tumors (ELSTs). Visceral manifestations
include renal carcinomas and cysts, pancreatic neuroendocrine tumors and cysts …