People who have VHL disease may experience tumors and/or cysts in up to ten parts of the body, including the brain, eyes, kidney, and adrenal glands.
VHL disease is different in every patient, even within the same family. Since it is impossible to predict exactly how and when the disease will present for each person, it is very important to check regularly for possible VHL manifestations throughout a person’s lifetime.
Most of these VHL tumors are benign, but that does not mean they are problem-free! In fact, benign VHL tumors can still be very serious. As they grow in size, these tumors and the associated cysts can cause an increased pressure on the structure around them. This pressure can create symptoms including severe pain.
Some VHL tumors, such as those in the kidney and pancreas, can grow to a stage where they become cancerous. The goal is to identify these lesions when they are small before they spread to other parts of the body.
The Active Surveillance Guidelines focus on searching for manifestations that are often problematic. Notice, the Active Surveillance Guidelines do not include special monitoring of the reproductive tract, lung, and liver as these manifestations are often not a problem.
The Emergency Card that VHLA encourages VHL patients to carry, focuses on signs and symptoms associated with VHL manifestations.