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Paradoxical Response to Dexamethasone in the Diagnosis of Primary Pigmented Nodular Adrenocortical Disease

Constantine A. Stratakis, MD, DSc; Nicholas Sarlis, MD, PhD; Lawrence S. Kirschner, MD, PhD; J. Aidan Carney, MD, PhD; John L. Doppman, MD; Lynnette K. Nieman, MD; George P. Chrousos, MD; and Dimitris A. Papanicolaou, MD
[+] Article and Author Information

From the National Institutes of Health, Bethesda, Maryland; and the Mayo Clinic, Rochester, Minnesota.


Acknowledgments: The authors thank the nursing staff of the 8W and 9W wards of the NIH clinical center for their help and expert technical assistance in completing patient tests. They especially thank Barbara Filmore, who supervised performance of all hormonal assays, and the patients who participated in our clinical research protocol. They also thank Dr. David E. Schteingart of the University of Michigan for useful discussions of dexamethasone-related paradoxical response of urinary glucocorticoids and for a critical review of this work.

Grant Support: By intramural funds of the National Institute of Child Health and Human Development for clinical research protocol 95-CH-059 (Dr. Stratakis, principal investigator).

Requests for Reprints: Constantine A. Stratakis, MD, DSc, National Institutes of Health, Building 10, Room 10N262, 10 Center Drive, MSC 1862, Bethesda, MD 20892-1862; e-mail, stratakc@cc1.nichd.nih.gov. For reprint orders in quantities exceeding 100, please contact the Reprints Coordinator; phone, 215-351-2657; e-mail, reprints@mail.acponline.org.

Current Author Addresses: Drs. Stratakis, Sarlis, Kirschner, Nieman, Chrousos, and Papanicolaou: National Institutes of Health, Building 10, Room 10N262, 10 Center Drive, MSC 1862, Bethesda, MD 20892-1862.

Dr. Carney: Plummer N-10, Mayo Clinic, One South Drive, Rochester, MN 55905.

Dr. Doppman: Diagnostic Radiology Department, Warren Grant-Magnuson Clinical Center, 10 Center Drive, MSC 1182, Bethesda, MD 20892-1182.


Ann Intern Med. 1999;131(8):585-591. doi:10.7326/0003-4819-131-8-199910190-00006
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Primary pigmented nodular adrenocortical disease is a bilateral adrenal disorder that leads to adrenocorticotropin (ACTH)-independent cases of the Cushing syndrome. In most cases, this disease occurs as part of the Carney complex, an autosomal dominant, multiple neoplasia syndrome that consists of skin lentigines, myxomas, and other nonendocrine and endocrine tumors (1). Cardiac myxoma, a tumor that may cause stroke and death, is among the most frequent and is often the first manifestation of the Carney complex (12). The diagnosis of primary pigmented nodular adrenocortical disease should be followed by screening for the Carney complex and, in particular, its potentially fatal cardiac component (12).

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Figure 1.
Macronodular appearance on computed tomography of the adrenal gland in a patient with primary pigmented adrenocortical disease (left) and the more typical “bead-on-a-string” appearance of an adrenal gland in a patient with primary pigmented nodular adrenocortical disease (right).
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Figure 2.
Mean percentage change in 24-hour excretion of 17-hydroxycorticosteroids (17-OHCS) (top) and urinary free cortisol (bottom) during the Liddle test.PP

Circles represent patients with primary pigmented nodular adrenocortical disease, squares represent patients with macronodular adrenocortical disease, and triangles represent patients with single adenomas. The baseline value for both steroids is the mean of 2 days of 24-hour urine collection (days 1 and 2 of the Liddle test). Error bars represent the SE. *Statistical significance for patients with primary pigmented nodular adrenocortical disease compared with patients with macronodular adrenocortical disease (Mann-Whitney U test, < 0.001 for all comparisons); +Statistical significance for patients with primary pigmented nodular adrenocortical disease compared with patients with a single adenoma (Mann-Whitney U test, < 0.01 for urinary free cortisol level and 17-hydroxycorticosteroid level on day 6 of the Liddle test).

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Figure 3.
Receiver-operating characteristic curves and urinary free cortisol levels during the Liddle test. Top.solid linesdotted linesBottom.

Receiver-operating characteristic curves for urinary free cortisol level ( ) and 17-hydroxycorticosteroid level ( ) in the diagnostic evaluation of primary pigmented nodular adrenocortical disease compared with other primary adrenal disorders during the Liddle test. The upper two curves represent urinary steroid values on day 6 of the Liddle test, and the lower two curves represent baseline urinary steroid values. Urinary free cortisol levels. MAD = macronodular adrenocortical disease; PPNAD = primary pigmented nodular adrenocortical disease.

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Summary for Patients

Diagnosing a Rare but Potentially Deadly Disease of the Adrenal Glands

The summary below is from the full report titled “Paradoxical Response to Dexamethasone in the Diagnosis of Primary Pigmented Nodular Adrenocortical Disease.” It is in the 19 October 1999 issue of Annals of Internal Medicine (volume 131, pages 585-591). The authors are C.A. Stratakis, N. Sarlis, L.S. Kirschner, J.A. Carney, J.L. Doppman, L.K. Nieman, G.P. Chrousos, and D.A. Papanicolaou.

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