Pancreatic islet cell tumor associated with Cushing's syndrome.
Seven female patients with a pancreatic islet cell tumor and Cushing's syndrome are described. Five patients initially had symptoms and signs of hypercortisolism, and two presented with an abdominal mass and had subsequent development of the syndrome. The tumors ranged in size from 2 to 12 cm in diameter, and all were argyrophilic with the Grimelius technique. Four cases had positive immunostaining for adrenocorticotropic hormone; other hormones were identified in four cases. Six of the patients were dead at the time of this report: five with metastatic tumor within 4 years of diagnosis and one 19 years after diagnosis with hepatic metastases of undetermined origin. One patient was alive with hepatic metastases 20 years after operation. Among the 42 known cases of pancreatic islet cell tumor and Cushing's syndrome, the 5-year survival was 16%.
D000182:ACTH Syndrome, Ectopic; D007516:Adenoma, Islet Cell / Q000378:metabolism* / Q000401:mortality / Q000473:pathology; D000293:Adolescent; D000328:Adult; D003480:Cushing Syndrome / Q000209:etiology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007124:Immunoenzyme Techniques; D008875:Middle Aged; D009362:Neoplasm Metastasis; D010190:Pancreatic Neoplasms / Q000378:metabolism* / Q000401:mortality / Q000473:pathology; D009384:Paraneoplastic Endocrine Syndromes