Von Hippel-Lindau Syndrome
Von Hippel-Lindau syndrome (VHL) causes multiple tumors to form throughout the body, many of which are vascular. Although most of the tumors associated with VHL are non-cancerous, they can cause damage.
There is high risk for the following tumors:
- Adrenal gland tumors (pheochromocytomas)
- High blood pressure and otherwise unexplainable sweating and anxiety
- Brain and spinal hemangioblastomas
- Abnormal growth of blood vessels; symptoms include headaches, vomiting, dizziness, and irregular gait
- Ear tumors (endolymphatic sac tumors)
- May cause hearing loss
- Kidney cancer
- Clear cell type
- Non-cancerous cystadenomas of the epididymis
- Occurs in males
- Pancreatic tumors and cysts
- Generally do not cause symptoms unless they press on other organs
- Retinal hemangioblastomas
- Usually do not cause symptoms; small risk of vision loss
VHL is caused by mutations in the VHL gene. Our cancer genetic counselors can determine whether genetic testing for VHL is appropriate for you. Identification of a person’s VHL mutation is the first step in alleviating disease progression.
Most people with VHL also have a parent with VHL. Each child of a person with VHL has a 50 percent risk to also have VHL. Genetic testing can be used to determine which family members have VHL.