Why choose us for von Hippel-Lindau syndrome care?
Von Hippel-Lindau syndrome is a rare genetic problem that causes tumors, some cancerous, to form in multiple organ systems. At Mays Cancer Center, home to UT Health San Antonio MD Anderson Cancer Center, you have access to pediatric and adult specialists with experience treating this complex condition.
Von Hippel-Lindau syndrome is one of many rare genetic endocrine tumors we treat. Find out more about rare and familial endocrine tumors.
What you need to know about von Hippel-Lindau syndrome
- This condition occurs due to an abnormal change (mutation) in the von Hippel-Lindau gene. This gene normally keeps cells from dividing too fast and forming tumors. In patients with von Hippel-Lindau syndrome, the mutation prevents the gene from doing its job.
- Von Hippel-Lindau syndrome is challenging to diagnose because it can affect many organ systems, including your eyes and kidneys. The symptoms often mimic other medical problems.
- People with this condition need lifelong care. You may need surgery to remove tumors once they get to a certain size. Even after surgery, tumors might come back, or new ones may form in different areas.
How to talk to your doctor about von Hippel-Lindau syndrome
Von Hippel-Lindau syndrome is a complex condition that can affect your family’s health and your own. We take time to answer your questions and help you plan for future health needs.
You might want to ask your doctor about:
- Additional testing that may be necessary to check for tumors and how often you need them
- Diagnosis, including the size and location of tumors, if you have any, and whether they are cancer
- Genetic testing to determine whether other family members carry the von Hippel-Lindau syndrome gene mutation
- Treatments, if needed, which may include clinical trials, if eligible
- Support to help you and your family cope with the ways von Hippel-Lindau disease may affect your life
Making care decisions can be challenging. In addition to the support you receive from our team, your loved ones can also help. Feel free to bring family members or trusted friends with you to care appointments.
There are two types of von Hippel-Lindau syndrome:
- Type 1 has a low risk of a certain type of adrenal tumor (pheochromocytoma)
- Type 2 includes pheochromocytoma with other tumor types
Both types increase your risk of different types of tumors and cancer, including:
- Hemangioblastoma, a benign brain tumor that can form in the brain, spinal cord and back of the eye
- Kidney cancer
- Retinal hemangioblastoma, a type of eye tumor
- Neuroendocrine tumors of the pancreas