Von Hippel-Lindau syndrome (VHL) is characterized by an increased risk of developing the tumors listed below:
Hemangioblastomas (benign, or noncancerous, tumors made up of nests of blood vessels) of the brain and spine
Hemangioblastomas of the retina
Pheochromocytomas (a tumor of the chromaffin cells, which are present in the adrenal gland; usually benign, or noncancerous) within or outside of the adrenal gland
Renal cell carcinoma (cancerous tumor of the kidney)
Less commonly, some individuals develop endolymphatic sac tumors (ear tumors that can cause deafness if undetected), pancreatic tumors, and cystadenomas of the epididymis or broad ligament. Other manifestations include cysts (pockets of fluid) of the kidney and pancreas.