The cause of von Hippel-Lindau is hereditary. Von Hippel-Lindau disease causes tumors to form in the kidneys, brain, eye, brainstem, spinal cord, adrenal glands, and pancreas. Those with Von Hippel-Lindau disease have an increased risk for cancer, especially kidney cancer.

Those with von Hippel-Lindau disease have:

• 15% have tumors of the spinal cord
• 23% have kidney cancer
• 30% have tumors of the adrenal gland
• 50% have abdominal cysts: kidney, pancreas
• 54% have tumors of the cerebellum
• 57% have tumors of the retina