von Hippel-Lindau disease: a genetic condition predisposing tumor formation

Von Hippel-Lindau disease (VHL) is a rare, autosomal, dominantly transmitted disease that genetically predisposes affected people to tumor development. The most common manifestations of the disease and those associated with the greatest morbidity and mortality are cerebellar, spinal, and medullary hemangioblastomas, retinal angiomas, renal cell carcinomas, and pheochromocytomas. In this paper the genetic transmission, tumorigenesis, diagnosis, treatment, surveillance methods, and nursing implications associated with VHL are reviewed. The importance of early detection and the need for long-term follow-up are emphasized. Oncology nurses will need to stay abreast of the genetic aspects of cancer as information in this area increases and as expanded roles bring them in closer contact with patients and families affected by these conditions.