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Hypodense Nodularity on Computed Tomography: Novel Imaging and Pathology of Micronodular Adrenocortical Hyperplasia Associated with Myelolipomatous Changes

Department of Diagnostic Radiology (N.A.C., N.J.P.), Warren Grant Magnuson Clinical Center, National Institutes of Health (NIH), Bethesda, Maryland 20892; Section on Endocrinology and Genetics (N.A.C., K.G., M.K., C.A.S.), Developmental Endocrinology Branch, National Institute of Child Health and Human Development, Bethesda, Maryland 20892; Laboratory of Pathology (D.C.), National Cancer Institute, NIH, Bethesda, Maryland 20892; Pediatric Endocrinology (J.L.R.), Thomas Jefferson University, Philadelphia, Pennsylvania 19107; and Department of Laboratory Medicine and Pathology (J.A.C., emeritus member), Mayo Clinic, Rochester, Minnesota 55905

Address all correspondence and requests for reprints to: Constantine A. Stratakis, M.D., D.Sc., Section on Endocrinology and Genetics, Developmental Endocrinology Branch, National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 10N262, 10 Center Drive, MSC 1862, Bethesda, Maryland 20892-1862. E-mail: stratakc{at}cc1.nichd.nih.gov.

An 8.5-yr-old white girl with a history of weight gain, mood changes, and early menarche presented for the evaluation of possible Cushing syndrome. Clinical examination revealed mild hypertension, facial plethora, palpable supraclavicular fat pads, and a dorsal fat pad. There were no skin pigmentation changes suggestive of ACTH excess or pigmented spots that would indicate Carney complex (CNC) (1). Family history was essentially noncontributory.

Urinary free cortisol and 17-OH-steroid levels were elevated at baseline and responded paradoxically to dexamethasone administration during Liddle’s test (2). Computed tomography (CT) examination of the adrenal glands demonstrated bilateral adrenal enlargement, the left gland being larger than the right (Fig. 1). After contrast enhancement, many small, hypodense, nonenhancing nodules were seen in both glands; the largest (12 mm in diameter) was on the left side (Fig. 2, A and B). Three well-delineated, round or ovoid (4, 5, and 12 mm in diameter) lesions with density similar to fat were also demonstrated in the right adrenal (not shown). These features have not been seen before in the imaging of adrenocortical hyperplasia (3, 4).

View larger version (132K): [in this window] [in a new window]   FIG. 1. Precontrast CT imaging shows bilateral adrenocortical hyperplasia (arrows), with the left adrenal gland being larger than the right.

View larger version (73K): [in this window] [in a new window]   FIG. 2. A, CT imaging after injection of a contrast agent and with a slice thickness of 3 mm revealed an enlarged left adrenal gland with a well-delineated multinodular contour. Several hypodense nodules, from 2 to 9 mm in diameter, were also seen (arrows). Histological examination showed that all hypodense nodules corresponded to cortical areas with myeloid metaplasia. B, In the left limb of the left adrenal, a well-delineated ovoid lesion with a greatest diameter of 12 mm and a density similar to fat is seen.

View larger version (71K): [in this window] [in a new window]   FIG. 3. A, Hematoxylin and eosin-stained sections of the right adrenal showed micronodular hyperplasia with myeloid metaplasia and without pigmentation (magnification, x20). B, Cortical nodular cells stained positive for synaptophysin (magnification, x100).

We conclude that myelolipomatous changes in the nodules of micronodular adrenocortical hyperplasia can make the CT diagnosis of this disorder difficult. The characteristic images are shown in Figs. 1 and 2.

It is unclear at this point, given the negative genetic testing, whether the presented patient indeed had PPNAD or an unrecognized form of micronodular adrenocortical hyperplasia associated with corticotropin-independent Cushing syndrome.

	Footnotes

 
Abbreviations: CNC, Carney complex; CT, computed tomography; PPNAD, primary pigmented nodular adrenocortical disease.

Received January 13, 2004.

Accepted April 27, 2004.

	References

Top References

 

1. Stratakis CA, Kirschner LS, Carney JA 2001 Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab 86:4041–4046[Abstract/Free Full Text]
2. Stratakis CA, Sarlis N, Kirschner LS, Carney JA, Doppman JL, Nieman LK, Chrousos GP, Papanicolaou DA 1999 Paradoxical response to dexamethasone in the diagnosis of primary pigmented nodular adrenocortical disease. Ann Intern Med 131:585–591[Abstract/Free Full Text]
3. Doppman JL, Travis WD, Nieman L, Miller DL, Chrousos GP, Gomez MT, Cutler GB, Loriaux DL, Norton JA 1989 Cushing syndrome due to primary pigmented nodular adrenocortical disease: findings at CT and MR imaging. Radiology 172:415–420[Abstract/Free Full Text]
4. Young Jr WF, Carney JA, Musa BU, Wulffraat NM, Lens JW, Drexhage HA 1989 Familial Cushing’s syndrome due to primary pigmented nodular adrenocortical disease: reinvestigation 50 years later. N Engl J Med 321:1659–1664[Medline]
5. Stratakis CA, Carney JA, Kirschner LS, Willenberg HS, Brauer S, Ehrhart-Bornstein M, Bornstein SR 1999 Synaptophysin immunoreactivity in primary pigmented nodular adrenocortical disease: neuroendocrine properties of tumors associated with Carney complex. J Clin Endocrinol Metab 84:1122–1128[Abstract/Free Full Text]
6. Sandrini F, Stratakis CA 2003 Clinical and molecular genetics of Carney complex. Mol Genet Metab 78:83–92[CrossRef][Medline]
7. Groussin L, Jullian E, Perlemoine K, Louvel A, Leheup B, Luton JP, Bertagna X, Bertherat J 2002 Mutations of the PRKAR1A gene in Cushing’s syndrome due to sporadic primary pigmented nodular adrenocortical disease. J Clin Endocrinol Metab 87:4324–4329[Abstract/Free Full Text]

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