Ectopic ACTH syndrome due to salivary gland adenoid cystic carcinoma.
A 68-year-old woman developed Cushing syndrome associated with a submaxillary salivary gland adenoid cystic carcinoma. High levels of immunoreactive adrenocorticotrophic hormone (ACTH) were found in the tumor and peripheral blood. Urinary 17-hydroxycorticosteroid levels decreased following administration of dexamethasone, suggesting possible suppression of ectopic ACTH production. however, there was no specific in vitro binding of dexamethasone by tumor cytosol. A trial of metyrapone therapy resulted in control of hypokalemia and hyperglycemia. Both plasma cortisol and 11-deoxycortisol levels decreased with metyrapone administration, suggesting that this agent may inhibit not only 11-beta-hydroxylation, but also possibly earlier steps in glucocorticoid synthesis.
D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D000368:Aged; D001344:Autopsy; D003528:Carcinoma, Adenoid Cystic / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism; D003480:Cushing Syndrome / Q000209:etiology*; D005260:Female; D006729:Hormones, Ectopic / Q000378:metabolism; D006801:Humans; D008797:Metyrapone / Q000627:therapeutic use; D009362:Neoplasm Metastasis; D009384:Paraneoplastic Endocrine Syndromes / Q000188:drug therapy; D012468:Salivary Gland Neoplasms / Q000150:complications* / Q000188:drug therapy / Q000378:metabolism
D006729:Hormones, Ectopic; D000324:Adrenocorticotropic Hormone; D008797:Metyrapone