Guidelines
These are the current guidelines offered by the VHL alliance for surveillance studies of VHL patients.
Update your location to show providers, locations, and services closest to you.
Von Hippel-Lindau syndrome (VHL) is a hereditary condition associated with tumors arising in multiple organs. VHL-related tumors include hemangioblastomas, which are blood vessel tumors of the brain, spinal cord, and retina. The retinal tumors are also called retinal angiomas, which can lead to blindness if not treated in a timely manner. People with VHL also have an increased risk of developing clear cell renal cell carcinoma (ccRCC), which is a specific type of kidney cancer, as well as a type of tumor in the pancreas known as pancreatic neuroendocrine tumor (pNET). Tumors of the adrenal gland or pheochromocytoma can also develop, with a small number becoming metastatic, meaning they spread to other parts of the body.
It is estimated that about 1 in 30,000 people has VHL. About 10% of people with VHL do not have any family history of the condition. They have a de novo mutation, meaning a new mutation in the VHL gene not present in their parents.
On August 13, 2021, the US Food and Drug Administration (FDA) approved Belzutifan [pronounced bell-ZOO-ti-fan] (Welireg) for the treatment of:
The medication is now available to be prescribed in the US.
These are the current guidelines offered by the VHL alliance for surveillance studies of VHL patients.