This condition causes tumors throughout your body, which means you may have several different symptoms depending on where tumors develop and the tumors’ sizes. Symptoms may include: VSports在线直播.
von Hippel-Lindau syndrome is an inherited disorder that may happen when one of your biological parents passes on an abnormal version of the VHL gene, a tumor suppressor gene that manages cell growth. V体育2025版.
These genes make special proteins that put the brakes on cell growth that may cause cancer. When tumor suppressor genes mutate (change), it’s as if they switch from pressing on the brakes to hitting the gas pedal, suddenly putting cell growth into overdrive.
This disorder follows an autosomal dominant inheritance pattern. That means if you inherit an abnormal VHL gene from one of your biological parents, there’s a 50% chance that you’ll have von Hippel-Lindau disease. That being said, research shows up to 10% of people with VHL don’t have a family history of the disorder.
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Healthcare providers may suspect you have VHL if you have symptoms of one of the conditions that von Hippel-Lindau disease may cause, like a hemangioblastoma or clear cell renal carcinoma. But genetic testing is the only way to confirm that you have the condition. If anyone in your family has von Hippel-Lindau disease, ask your healthcare provider if genetic testing is right for you.
Treatments vary depending on the type of tumor or cyst that VHL causes. Your healthcare provider will explain your treatment options. Common treatments are:
von Hippel-Lindau disease doesn’t have a cure. The goal of treatment is to find and remove tumors as early as possible. Your provider may recommend surgery to remove tumors along with other treatments.
If you have this condition, you may have regular tests to monitor for signs of specific kinds of diseases. Finding and treating tumors early on may reduce the impact that von Hippel-Lindau syndrome may have on your life.
No, it can’t. von Hippel-Lindau disease occurs when a biological parent passes a genetic mutation onto their child. If you have a family history of VHL, genetic tests will determine whether you’re carrying the mutation.
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While you can’t prevent von Hippel-Lindau disease, it’s helpful to know your risk and understand how VHL may affect you. If you know you’re at risk, healthcare providers can watch for signs of VHL-related tumors and move quickly to remove those tumors.
If you carry the VHL gene and plan to have a baby, a genetic counselor can help you understand the risks of passing the gene to your child.
There aren’t any screening guidelines, like the cancer screening tests set by the U.S. Preventive Services Task Force. If genetic tests show you carry an abnormal VHL gene, your healthcare team may recommend that you have specific tests that may detect issues early on. For example, your provider may recommend that you have an abdominal magnetic resonance imaging (MRI) scan every two years to check for VHL-related cancers, like clear cell renal carcinoma.
Living with VHL can mean living with uncertainty. If you inherit the genetic mutation that causes von Hippel-Lindau disease, there’s a 50% chance you’ll have the condition. If you do, there’s a 97% chance you’ll have certain kinds of cancer and other serious illnesses.
But no one can predict exactly how VHL may affect your life. Here are some things you can do that may help you manage those challenges:
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You should call your healthcare provider if you experience:
You’ve just learned you have a family history of von Hippel-Lindau disease. Here are some questions you may want to ask your healthcare provider:
von Hippel-Lindau disease (VHL) is a rare genetic disorder. It affects 1 in 36,000 people. Learning you’re the 1 in 36,000 may come as a shock, especially if you don’t have a family history of the disorder.
If genetic tests show you have VHL, give yourself some space to absorb the news, and then take time to understand what your diagnosis means. For example, you may have questions about your risk of developing different kinds of cancer and tumors or what your diagnosis means for the rest of your family. Don’t hesitate to ask your healthcare team what you can expect. And ask for help managing the emotional stress that living with VHL can cause.
Cleveland Clinic offers the latest cancer genetic counseling and testing to help you understand your cancer risk and make the most of your health.
Last reviewed on 02/12/2024.
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