Somatostatinoma of the pancreas associated with von Hippel-Lindau disease.

A 39-year-old man was admitted because of lumbago, vomiting and massive gastrointestinal bleeding. Oliguria developed a few days later, which was followed by hyperkalemia and cardiac arrest. Autopsy disclosed multiple renal cell carcinomas with diffuse metastasis to the liver, adrenal gland, psoas muscle and vertebrae. In addition, a somatostatinoma was found in the pancreas. From these findings and past history of cerebellar hemangioblastoma and spinal hemangioma he was diagnosed to have von Hippel-Lindau disease. Von Hippel-Lindau disease with islet cell tumor is very rare and is reported here with a review of literature.