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October 1986

Cushing's Syndrome Resulting From Primary Pigmented Nodular Adrenocortical Disease

Author Affiliations

From the Departments of Pediatrics (Dr Kaplowitz), Pathology (Dr Carpenter), Surgery (Dr Newsome), and Medicine (Dr Downs), Medical College of Virginia, Virginia Commonwealth University, Richmond.

Am J Dis Child. 1986;140(10):1072-1075. doi:10.1001/archpedi.1986.02140240118038

• In a 10-year-old boy with Cushing's syndrome, the dexamethasone suppression test, the metyrapone test, and both basal and corticotropin-releasing factor–stimulated corticotropin levels all indicated a primary adrenal disorder. However, a computed tomographic scan failed to detect an adrenal tumor. At surgery, the adrenal glands were not enlarged but were studded with small pigmented nodules composed of enlarged nonmalignant adrenocortical cells. This unusual abnormality, referred to as primary pigmented nodular adrenocortical disease, is associated with autonomous hypersecretion of cortisol primarily in children and young adults. Our patient was cured by total bilateral adrenalectomy and corticosteroid replacement therapy, the treatment of choice for this condition.

(AJDC 1986;140:1072-1075)