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CARL D. MALCHOFF, JOSEPH ROSA, C. ROWAN DEBOLD, ROBERT A. KOZOL, GALE R. RAMSBY, DAVID L. PAGE, DIANA M. MALCHOFF, DAVID N. ORTH, Adrenocorticotropin-Independent Bilateral Macronodular Adrenal Hyperplasia: An Unusual Cause of Cushing’s Syndrome, The Journal of Clinical Endocrinology & Metabolism, Volume 68, Issue 4, 1 April 1989, Pages 855–860, https://doi.org/10.1210/jcem-68-4-855
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Abstract
Inappropriate ACTH secretion with bilateral diffuse or macronodular adrenal hyperplasia is the most common cause of Cushing’s syndrome. This report describes a patient with Cushing’s syndrome and feminization due to ACTH-independent bilateral macronodular adrenal hyperplasia. A 47-yrold black man presented with Cushingoid features, diabetes mellitus, hypertension, impotence, and gynecomastia. Urinary cortisol and 17-hydroxycorticosteroid excretion were 94 nmol/mmol creatinine (normal, <32) and 5.8 μmol/mmol creatinine (normal, 0.6–3.6), respectively. Both decreased by less than 30% after administration of dexamethasone (8 and 16 mg/day), and urinary 17-hydroxycorticosteroid excretion did not increase after metyrapone (750 mg, orally, every 4 h for six doses). Plasma ACTH was undetectable (<1 pmol/L) and was not stimulated by administration of metyrapone or ovine CRH. Serum testosterone was 5.2 nmol/L (normal, 7–30), FSH was 5 U/L (normal, 3–18), LH was 2.8 U/L (normal, 1.5–9.2), and estrone was 767 pmol/L (normal, 55–240). Both adrenal glands were enlarged, with a total weight of 86 g (normal, 8–10), and contained multiple nodules (diameter, >0.5 cm) composed of two active cell types, one of which was also observed between the nodules. Cushing’s syndrome with feminization due to ACTH-independent bilateral macronodular adrenal hyperplasia is an unusual process of unknown etiology that should be included with the other known causes of Cushing’s syndrome.
