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January 1977

Ectopic ACTH Syndrome Due to Salivary Gland Adenoid Cystic Carcinoma: Response to Metyrapone

Author Affiliations

From the Veterans Administration Hospital, St Louis, and the Department of Internal Medicine, St Louis University School of Medicine.

Arch Intern Med. 1977;137(1):102-105. doi:10.1001/archinte.1977.03630130070017

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-β-hydroxylation, but also possibly earlier steps in glucocorticoid synthesis.

(Arch Intern Med 137:102-105, 1977)

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