Von-Hippel Lindau disease


vHL is a rare genetic disorder characterized by the development of both benign and malignant tumors in various systems, most notably within the CNS and in the abdomen.

The main features can be recalled using the mnemonic HIPPEL (taken from the article on vHL):

  • Haemangioblastoma
  • Increased risk of RCC
  • Phaeochromocytoma
  • Pancreatic lesions (cysts, cystadenomas, cystadenocarcinomas)
  • Eye dysfunction (retinal hemangioblastomas)
  • Liver, renal and pancreatic cysts