vhl alliance

What is VHL?

VHL disease is a genetic condition characterized by blood vessel tumors in up to ten areas of the body. These tumors can be benign or cancerous.

VHL, or von Hippel-Lindau, is named after two physicians that described these tumors in different parts of the body. Eugene von Hippel was an ophthalmologist who first described these tumors in the eye, and Arvid Lindau described them in the brain and spine. Today we understand that these manifestations, as well as manifestations in 7 other areas of the body, are caused by a flaw in one gene, the VHL gene.

Since it is impossible to predict exactly how the disease will present or progress in a particular person, regular surveillance is extremely important for people living with VHL.

The Active Surveillance Guidelines were developed to make sure VHL tumors can be found and managed before they cause irreparable damage to the body. With careful monitoring, early detection, and appropriate treatment, the most harmful consequences of this gene mutation can be greatly reduced, or in some cases, completely prevented.

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