[von Hippel-Lindau disease].

Abstract Von Hippel-Lindau disease is a rare autosomal dominant hereditary tumor syndrome. The most common manifestations of the disease include cerebellar and spinal hemangioblastomas, retinal angiomas, renal cysts and clear cell cancer, pheochromocytoma, tumors of the inner ear, pancreatic cysts and neuroendocrine tumors. Coordination of examinations and treatment as well as collaboration between specialties is important. Screening of family members is recommended, and those having VHL should be monitoried annually through the rest of their lives.