Adrenocorticotropin-independent bilateral macronodular adrenal hyperplasia: An unusual cause of cushing’s syndrome

Carl D. Malchoff, Joseph Rosa, C. Rowan Debold, Robert A. Kozol, Gale R. Ramsby, David L. Page, Diana M. Malchoff, David N. Orth

Research output: Contribution to journalArticle

74 Scopus citations

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-yr-old 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 pmol/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.

Original languageEnglish (US)
Pages (from-to)855-860
Number of pages6
JournalJournal of Clinical Endocrinology and Metabolism
Volume68
Issue number4
DOIs
StatePublished - Apr 1989

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Endocrinology
  • Clinical Biochemistry
  • Biochemistry, medical

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    Malchoff, C. D., Rosa, J., Debold, C. R., Kozol, R. A., Ramsby, G. R., Page, D. L., Malchoff, D. M., & Orth, D. N. (1989). Adrenocorticotropin-independent bilateral macronodular adrenal hyperplasia: An unusual cause of cushing’s syndrome. Journal of Clinical Endocrinology and Metabolism, 68(4), 855-860. https://doi.org/10.1210/jcem-68-4-855