Evidence for ectopic ACTH production years after bilateral adrenalectomy for Cushing's syndrome: in vivo and in vitro studies.
Three patients presented with hyperpigmentation and high plasma levels of ACTH 4-5 years after bilateral adrenalectomy for Cushing's "disease". X-rays and ct-scan of the lungs showed a small pulmonary mass in all. ACTH levels (1100-2000 pg/ml) were not suppressible by high doses hydrocortisone. A carcinoid tumor was removed in two cases and a chemodectoma in the third. Evidence for ACTH secretion by these tumors was provided by both decrease of ACTH levels after surgery and/or in vitro studies. Both carcinoid cultures showed a basal ACTH production, which was clearly increased by LVP (10(-7) M) and CRF (10(-7) M). Our findings in vitro studies suggest the presence of specific receptor sites for physiological stimuli (LVP and CRF) in tumor cell membranes.
D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D000328:Adult; D002276:Carcinoid Tumor / Q000503:physiopathology; D003346:Corticotropin-Releasing Hormone / Q000494:pharmacology; D003480:Cushing Syndrome / Q000503:physiopathology* / Q000601:surgery; D005260:Female; D006729:Hormones, Ectopic / Q000378:metabolism*; D006801:Humans; D066298:In Vitro Techniques; D008175:Lung Neoplasms / Q000503:physiopathology; D008236:Lypressin / Q000494:pharmacology; D008297:Male; D010236:Paraganglioma, Extra-Adrenal / Q000503:physiopathology; D009384:Paraneoplastic Endocrine Syndromes / Q000503:physiopathology
D006729:Hormones, Ectopic; D008236:Lypressin; D000324:Adrenocorticotropic Hormone; D003346:Corticotropin-Releasing Hormone