Teaching Von Hippel-Lindau

Solapas principales


Maranha L.1, Da Silva L.2, Araújo J.3



The von Hippel-Lindau, tribute to dr. Eugen von Hippel and dr. Arvid Lindau that described retinal angiomas and central nervous system (CNS), respectively, is a relatively rare genetic disease (1 case in every 39 million people) that manifests with multiple systemic tumors. There is no difference between genders, and life expectancy is about 50 years old. Hemangioblastoma is the typical tumor of CNS in this disease, and with renal carcinoma are the major causes of mortality. This paper reviews some clinical, pathophysiological, epidemiological, surgical and genetic syndrome aspects, based in large series and productions of world literature.


Keywords: Von Hippel-Lindau; hemangioblastoma; neurocutaneous diseases


  1. Neurocirurgiã pelo Hospital de Clínicas – Universidade Federal do Paraná (HC-UFPR); Fellow de Neurorradiologia Intervencionista no Hospital Universitário Cajuru – Pontifícia Universidade Católica do Paraná (PUC-PR)
  2. Neurocirurgião do HC-UFPR
  3. Chefe do Serviço de Neurocirurgia do HC-UFPR

Serviço de Neurocirurgia - Hospital de Clínicas
Universidade Federal do Paraná
Curitiba - PR