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.
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Madigan Army Medical Center, Ft. Lewis, Tacoma, Washington.
D000328:Adult; D006801:Humans; D008297:Male; D006623:von Hippel-Lindau Disease / Q000175:diagnosis* / Q000628:therapy*