Successful Metyrapone Therapy of the Ectopic ACTH Syndrome

Raymond Coll, MB, BCh; Israel Horner, MB, BCh; Zaki Kraiem, AHWC; Joseph Gafni, MD
Arch Intern Med. 1968;121(6):549-553. doi:10.1001/archinte.1968.03640060063012.
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Over 100 cases of bilateral adrenocortical hyperplasia associated with nonendocrine malignancies have been reported. The neoplasm was most commonly of the bronchus; although a variety of primary sites has been recorded. Accordingly, nearly all of the cases were over 40 years of age.

The diagnostic term "ectopic ACTH syndrome" was applied following the identification of an adrenocorticotrophic hormone (ACTH)-like substance in the plasma and tumor tissue extracts of such patients.1-3 The syndrome was dominated by the profound biochemical disturbances of induced hyperadrenocorticism, namely diabetes mellitus, hypokalemic alkalosis, weakness, and muscle wasting, and heralded a relatively rapid fatal outcome. The classical physical stigmata of Cushing's syndrome were decidely uncommon.

It is the purpose of this paper to describe a case which is remarkable for the youth of the patient, the appearance of a full-blown Cushing's syndrome, and the prolonged favorable response to a cortisol inhibitor.

Patient Summary  A 16-year-old school boy


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