Von Hippel-Lindau Disease

Rare; approximate incidence is 1:36,000.

Usually occurs in young adults with complex multiple manifestations.

Pts with cerebral hemangioblastoma have a 23% incidence of VHLD; assess other systems carefully.

Space-occupying central nervous tumors (retinal and cerebellar hemangioblastomas in 60% of pts).

Pheochromocytoma (7–20% pts) may be undiagnosed.

Pregnancy and childbirth may dramatically change disease progression and symptom expression; multidisciplinary involvement essential.

VHLD is a complex multisystem disorder, and pts frequently require anesthesia for surgical treatment of tumors and embolizations.

Most common causes of death are renal cell carcinoma or complications from cerebral hemangioblastomas.