Von Hippel-Lindau disease in an adolescent

Von Hippel-Lindau disease is a hereditary neoplastic syndrome that generally manifests in early adulthood but does present occasionally in adolescence. In the past, diagnosis and management of this disorder fell within the domain of internists and surgeons. Because pediatricians are now seeing older patients, they must learn to recognize the various components of this disorder. Additionally, they must be able to assure appropriate medical evaluation and follow-up and also arrange for genetic counseling. Pediatricians already attuned to the benefits of preventive medicine should find themselves uniquely qualified to provide the level of care that this disease requires.