What is Von Hippel-Lindau disease (VHL)?
Von Hippel-Lindau disease (VHL) is a rare disease that causes tumors and cysts to grow in your body. They can grow in your brain and spinal cord, kidneys, pancreas, adrenal glands, and reproductive tract. The tumors are usually benign (non-cancerous). But some tumors, such as those in the kidney and pancreas, can become cancerous.
What causes Von Hippel-Lindau disease (VHL)?
Von Hippel-Lindau disease (VHL) is a genetic disease. It is inherited, which means that it is passed down from parent to child.
What are the symptoms of Von Hippel-Lindau disease (VHL)?
Symptoms of VHL depend on the size and location of the tumors. They may include
- Problems with balance and walking
- Weakness of the limbs
- Vision problems
- High blood pressure
How is Von Hippel-Lindau disease (VHL) diagnosed?
Detecting and treating VHL early is important. Your health care provider may suspect that you have VHL if you have certain patterns of cysts and tumors. There is a genetic test for VHL. If you have it, you will need other tests, including imaging tests, to look for tumors and cysts.
What are the treatments for Von Hippel-Lindau disease (VHL)?
Treatment can vary, depending on the location and size of the tumors and cysts. It usually involves surgery. Certain tumors may be treated with radiation therapy. The goal is to treat growths while they are small and before they do permanent damage. You will need to have careful monitoring by a doctor and/or medical team familiar with the disorder.
NIH: National Institute of Neurological Disorders and Stroke
- Genetics Home Reference: von Hippel-Lindau syndrome (National Library of Medicine)
- ClinicalTrials.gov: von Hippel-Lindau Disease (National Institutes of Health)
Journal Articles References and abstracts from MEDLINE/PubMed (National Library of Medicine)
- Article: Retinal capillary hemangioblastoma associated with retinochoroidal coloboma in Von Hippel-Lindau disease.
- Article: 68Ga DOTATATE PET/CT Versus 18F-FDG PET/CT for Detecting Intramedullary Hemangioblastoma in...
- Article: Adrenal adenoma in von Hippel-Lindau syndrome: A case report with review...
- Von Hippel-Lindau Disease -- see more articles