Von Hippel-Lindau Syndrome What is von Hippel-Lindau syndrome
(VHL)? Two eye doctors--von Hippel in Germany and Lindau in
Sweden--were the first to publish descriptions of tumors in
patients' eyes and brains, hallmarks of this genetic condition.
In the 1960s, the disease was named von Hippel-Lindau syndrome
to recognize their contributions in characterizing the
condition. Von Hippel-Lindau syndrome is a rare genetic disorder
characterized by an increased risk of developing the tumors
listed below: Hemangioblastomas. Benign (noncancerous) tumors
made up of nests of blood vessels of the brain and spine.
Hemangioblastomas of the retina Pheochromocytomas. A
neuroendocrine tumor, usually benign (noncancerous), within or
outside of the adrenal gland Renal cell carcinoma. Cancerous
tumor of the kidney that occurs in about 70 percent of
individuals with VHL. Less commonly, some individuals develop
endolymphatic sac tumors (ear tumors that can cause deafness if
undetected), pancreatic tumors, and cystadenomas of the e