Adrenocorticotropin-independent bilateral macronodular adrenal hyperplasia

An unusual cause of Cushing's syndrome

C. D. Malchoff, J. Rosa, C. R. DeBold, Robert Kozol, G. R. Ramsby, D. L. Page, D. M. Malchoff, D. N. Orth

Research output: Contribution to journalArticle

74 Citations (Scopus)

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
Pages (from-to)855-860
Number of pages6
JournalJournal of Clinical Endocrinology and Metabolism
Volume68
Issue number4
StatePublished - Jan 1 1989
Externally publishedYes

Fingerprint

Cushing Syndrome
Adrenocorticotropic Hormone
Hyperplasia
Feminization
17-Hydroxycorticosteroids
Medical problems
Gynecomastia
Hydrocortisone
Creatinine
Erectile Dysfunction
Diabetes Mellitus
Hypertension

ASJC Scopus subject areas

  • Biochemistry
  • Endocrinology, Diabetes and Metabolism

Cite this

Malchoff, C. D., Rosa, J., DeBold, C. R., Kozol, R., Ramsby, G. R., Page, D. L., ... 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.

Adrenocorticotropin-independent bilateral macronodular adrenal hyperplasia : An unusual cause of Cushing's syndrome. / Malchoff, C. D.; Rosa, J.; DeBold, C. R.; Kozol, Robert; Ramsby, G. R.; Page, D. L.; Malchoff, D. M.; Orth, D. N.

In: Journal of Clinical Endocrinology and Metabolism, Vol. 68, No. 4, 01.01.1989, p. 855-860.

Research output: Contribution to journalArticle

Malchoff, CD, Rosa, J, DeBold, CR, Kozol, R, Ramsby, GR, Page, DL, Malchoff, DM & Orth, DN 1989, 'Adrenocorticotropin-independent bilateral macronodular adrenal hyperplasia: An unusual cause of Cushing's syndrome', Journal of Clinical Endocrinology and Metabolism, vol. 68, no. 4, pp. 855-860.
Malchoff, C. D. ; Rosa, J. ; DeBold, C. R. ; Kozol, Robert ; Ramsby, G. R. ; Page, D. L. ; Malchoff, D. M. ; Orth, D. N. / Adrenocorticotropin-independent bilateral macronodular adrenal hyperplasia : An unusual cause of Cushing's syndrome. In: Journal of Clinical Endocrinology and Metabolism. 1989 ; Vol. 68, No. 4. pp. 855-860.
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