Recognition and management of von Hippel-Lindau disease.

Manifestations of von Hippel-Lindau disease include cerebellar tumors; renal, pancreatic and epididymal cysts; retinal angiomatosis; pheochromocytomas, and renal cell carcinoma. Patients who present with manifestations from one of the lesions associated with the disease are often incorrectly assumed to have an isolated lesion. First-degree relatives of a patient with von Hippel-Lindau disease have a 50 percent chance of developing the disease. Therefore, these individuals require close surveillance and careful management.