What's von Hippel-Lindau disease?
Von Hippel-Lindau disease is an inherited tumor-predisposition syndrome caused by changes in the VHL gene, producing benign and malignant tumors across several organs, especially the brain, retina, kidneys, and adrenal glands.
| Features mapped | 4 |
|---|---|
| Treatments mapped | 2 |
| Published sources | 3 |
| Last reviewed | 2026-08-04 |
Signs and symptoms
Renal cell carcinoma
People with von Hippel-Lindau disease have a high risk of clear cell renal cell carcinoma (a kidney cancer).
Cerebellar hemangioblastoma
Hemangioblastomas, benign blood-vessel tumors of the brain and spinal cord, are a hallmark of von Hippel-Lindau disease.
Retinal capillary hemangioma
Blood-vessel tumors can form in the retina (retinal hemangioblastomas) in von Hippel-Lindau disease and can threaten vision.
Adrenal pheochromocytoma
Pheochromocytomas, tumors of the adrenal gland that can raise blood pressure, occur in von Hippel-Lindau disease.
Treatment and management
What the research describes, not a recommendation. Treatment decisions belong with your clinician.
This covers treatments that appear in the published research mapped here. Investigational and experimental therapies are not included, so their absence is a boundary of this map, not a sign they do not exist.
belzutifan
Belzutifan is an FDA-approved medicine for von Hippel-Lindau disease that can treat VHL-associated tumors including renal cell carcinoma.
Used to help with: Renal cell carcinoma.
“Belzutifan's impact on patients with VHL syndrome, sporadic advanced ccRCC and pheochromocytoma/paraganglioma is discussed, three areas where belzutifan has obtained Food and Drug Administration approval.”
surgical resection
Surgical removal is the main treatment for hemangioblastomas in von Hippel-Lindau disease.
Used to help with: Cerebellar hemangioblastoma.
“The treatment of choice for hemangioblastomas is complete surgical resection.”
How to read the evidence labels
Where this comes from
This guide is built from 3 published source(s). Every claim above links back to one of them. Click any source ID to read the original on PubMed.
Take it further
Printed, source-linked documents built from this condition's graph — ready to bring to an appointment or attach to a coverage request. Every claim carries its published source, the same as this guide.