Growth hormone-releasing hormone (GHRH)-secreting pancreatic tumor in a patient with multiple endocrine neoplasia type I.
A growth hormone-releasing hormone (GHRH)-secreting pancreatic tumor in a 36-year-old man, who had typical, familial, multiple endocrine neoplasia (MEN) type I with hyperparathyroidism and acromegaly, is described. The resected tumor, weighing 30 g, showed unusual histological features characterized by a meningioma-like arrangement of crescent-shaped cells and contained many cells that reacted with C-terminal specific antibody to GHRH-44 and a few somatostatin-immunoreactive (IR) and calcitonin-IR cells, but no GH-IR cells. A high concentration of IR-GHRH (9.8-13.2 micrograms/g wet weight tissue) with the full molecular size of GHRH-44 was detected in a tumor extract. Electron immunocytochemical study by the protein A-gold method revealed GHRH-IR granules with a mean diameter of 147 nm. After removal of the tumor, the plasma IR-GHRH level became normal (decreasing from 299 to 16.1 pg/ml) and the plasma IR-GH level also decreased, but still remained slightly high (decreasing from 42.4 to 9.6 ng/ml), suggesting the presence of an adenomatous lesion in the hypophysis.
D000172:Acromegaly / Q000209:etiology; D000328:Adult; D013006:Growth Hormone / Q000378:metabolism; D013007:Growth Hormone-Releasing Hormone / Q000378:metabolism*; D006729:Hormones, Ectopic / Q000378:metabolism*; D006801:Humans; D008297:Male; D009377:Multiple Endocrine Neoplasia / Q000150:complications / Q000378:metabolism*; D010190:Pancreatic Neoplasms / Q000150:complications / Q000378:metabolism* / Q000648:ultrastructure; D013004:Somatostatin / Q000378:metabolism
D006729:Hormones, Ectopic; D013004:Somatostatin; D013006:Growth Hormone; D013007:Growth Hormone-Releasing Hormone