Von Hippel-Lindau (VHL)

Von Hippel-Lindau disease (VHL) is caused by a gene mutation which frequently induces both nonmalignant tumors and malignant tumors (or cancers) that can spread to other organs (become metastatic). Tumors may develop in up to ten different parts of the body. Many of these tumors involve the abnormal growth of blood vessels in different organs of the body. Most of these tumors are benign, meaning that they stay in the same organ where they began. However, VHL tumors in the kidney and pancreas can grow to a stage where they become “malignant,” meaning that the cancer can spread to other parts of the body.


Because VHL can cause malignant tumors, it is considered one of a group of familial cancer risk factors, which are transmitted genetically. The objective is to find tumors early, watch for signs that a tumor is growing, and to remove or disable the tumor before it invades other tissues. Benign tumors may also need treatment or removal if their growth will cause symptoms.