Metastatic islet cell tumor with ACTH, gastrin, and glucagon secretion. Clinical and pathologic studies with multiple therapies.
A patient with metastatic islet cell carcinoma demonstrated multiple clinical syndromes simultaneously with secretion of ACTH, gastrin, glucagon, and serotonin. Hepatic arterial embolization resulted in an initial decrease in all secretory products, which was sustained for glucagon and serotonin. Recrudescence of the Cushings and Zollinger-Ellison syndrome was managed by surgical extirpation of the primary tumor and regional metastases as well as bilateral adrenalectomy. Electron microscopy and immunocytochemistry of the primary tumor and the metastatic lesions revealed the presence of multiple types of granules within single cells and, different patterns of secretory profiles in different tumor sites.
D007516:Adenoma, Islet Cell / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000628:therapy; D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D000328:Adult; D003480:Cushing Syndrome / Q000503:physiopathology; D005260:Female; D005755:Gastrins / Q000378:metabolism*; D005934:Glucagon / Q000378:metabolism*; D006801:Humans; D008113:Liver Neoplasms / Q000556:secondary; D008854:Microscopy, Electron; D009362:Neoplasm Metastasis; D010190:Pancreatic Neoplasms / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000628:therapy; D014057:Tomography, X-Ray Computed
D005755:Gastrins; D000324:Adrenocorticotropic Hormone; D005934:Glucagon