VON HIPPEL-LINDAU SYNDROME

Course overview

VON HIPPEL-LINDAU (VHL) SYNDROME IS AN AUTOSOMAL DOMINANT DISEASE AFFECTING SEVERAL ORGAN SYSTEMS. THE DISEASE IS CHARACTERIZED BY THE GROWTH OF CYSTS AND/OR TUMORS. TUMORS CAN EITHER BE BENIGN OR MALIGNANT. THE CHARACTERISTIC TUMOR TYPE IN VHL IS THE HEMANGIOBLASTOMA, WHICH IS A BENIGN TUMOR COMPRISED OF NEWLY FORMED BLOOD VESSELS. HEMANGIOBLASTOMAS IN THE CENTRAL NERVOUS SYSTEM AND RETINA CAN CAUSE COMPLICATIONS, INCLUDING ATAXIA AND VISION LOSS. RENAL CELL CARCINOMA (RCC) AND PANCREATIC NEUROENDOCRINE TUMORS ARE ALSO SEEN IN PATIENTS WITH VHL. THIS ACTIVITY REVIEWS THE EVALUATION AND MANAGEMENT OF VON HIPPEL-LINDAU SYNDROME AND UNDERSCORES THE INTERPROFESSIONAL TEAM'S ROLE IN IMPROVING CARE FOR THE PATIENTS WITH THIS CONDITION.