Von Hippel-Lindau Syndrome
In general, treatment for kidney cancer is similar regardless of whether a patient has VHL. However, there is some evidence about specific VHL considerations regarding two types of treatment: surgery and targeted therapy. For a person with VHL and kidney cancer, surgery for a kidney tumor is generally considered when a tumor reaches three centimeters (cm) in size.
Targeted therapy is a treatment that targets the cancer’s specific genes, proteins, or the tissue environment that contributes to cancer growth and survival. Anti-angiogenesis therapy is a type of targeted therapy used in kidney cancer treatment. It is focused on stopping angiogenesis, which is the process of making new blood vessels. Because a tumor needs the nutrients delivered by blood vessels to grow and spread, the goal of anti-angiogenesis therapies is to “starve” the tumor. For people with VHL, there is emerging research showing that an anti-angiogenic drug called sunitinib (Sutent), which is classified as a tyrosine kinase inhibitor (TKI), may be effective against kidney cancer.
What are the screening options for VHL?
It is important to discuss with your doctor the following screening options, as each individual is different:
Yearly eye examination to look for retinal tumors, beginning around age 2
Yearly physical examination
Yearly 24-hour urine test to screen for elevated catecholamines, beginning around age 2
Yearly abdominal ultrasound (which uses sound waves to create a picture of the internal organs) to look at the kidneys, pancreas, and adrenal glands beginning in the teenage years; change screening to abdominal computed tomography (CT or CAT; creates a three-dimensional picture of the inside of the body with an x-ray machine) scan in adulthood
Magnetic resonance imaging (MRI; uses magnetic fields, not x-rays, to produce detailed images of the body) of the brain and spine every two years beginning in the teenage years
Screening options may change over time as new technologies are developed and more is learned about VHL. It is important to talk with your doctor about appropriate screening tests.