title: Ectopic Cushing's syndrome with periodic hormonogenesis--a case suggesting a pathogenetic mechanism.
abstract: We report on a case of ectopic Cushing's syndrome due to a thymic carcinoid tumour with periodic hormonogenesis. Periods of hormonal production averaged 27 days. Prior to bilateral adrenalectomy, mean (s.d) values of ACTH and cortisol were 202.1 (50.3) pg/ml and 46 (14.7) micrograms/dl, ACTH rising to 3996 +/- 425 pg/ml (P less than 0.01) and cortisol falling to 6.3 +/- 1.5 micrograms/dl (P less than 0.01) in the immediate postoperative period. During the late postoperative period (2-13 months following surgery) ACTH levels fell to 509.3 (123.8) pg/ml (P less than 0.01), but remaining even higher (P less than 0.01) than before adrenalectomy. The pattern of ACTH in the present case suggests the existence of a negative feedback exerted by the cortisol over tumoral ACTH.
authors.affiliation: Hospital Ramón y Cajal, Madrid, Spain.
authors.forename: V
authors.initials: V
authors.lastname: Estopiñán
authors.forename: C
authors.initials: C
authors.lastname: Varela
authors.forename: P
authors.initials: P
authors.lastname: Riobo
authors.forename: J R
authors.initials: JR
authors.lastname: Dominguez
authors.forename: J
authors.initials: J
authors.lastname: Sancho
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000097:blood / Q000150:complications*; D000315:Adrenalectomy; D000324:Adrenocorticotropic Hormone / Q000097:blood; D000328:Adult; D002276:Carcinoid Tumor / Q000097:blood / Q000150:complications*; D003480:Cushing Syndrome / Q000097:blood / Q000209:etiology* / Q000601:surgery; D005246:Feedback; D005260:Female; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D009384:Paraneoplastic Endocrine Syndromes / Q000150:complications*; D013953:Thymus Neoplasms / Q000097:blood / Q000150:complications*
chemical_list: D000324:Adrenocorticotropic Hormone; D006854:Hydrocortisone