title: Metastatic islet cell tumor in von Hippel-Lindau disease.
abstract: A 56-year-old woman with many unusual manifestations of von Hippel-Lindau syndrome is described. In addition to retinal hemangioblastomas, pheochromocytoma, renal cell carcinoma, and multiple organ cysts, she had a cerebellar astrocytoma, pancreatic exocrine insufficiency, diabetes mellitus, thyrotoxicosis, and a metastatic calcitonin-secreting islet cell carcinoma. This case report documents the first example of a metastatic islet cell tumor in a patient with von Hippel-Lindau disease. The possible relationship between this disorder, the other neurocutaneous syndromes, and the multiple endocrine neoplasia syndromes is discussed.
mesh_terms: D007516:Adenoma, Islet Cell / Q000473:pathology* / Q000556:secondary; D000310:Adrenal Gland Neoplasms / Q000473:pathology; D000798:Angiomatosis / Q000473:pathology*; D002116:Calcitonin / Q000096:biosynthesis; D005134:Eye Neoplasms / Q000473:pathology; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000473:pathology*; D013577:Syndrome; D006623:von Hippel-Lindau Disease / Q000473:pathology*
chemical_list: D002116:Calcitonin