The Use of Positron Emission Tomography-Computed Tomography ...

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Thomas George Oliver. Department of Medicine (P.B.B.), Nuclear Medicine Service (A.J.M.), and Endocrine Service (H.J.L., T.G.O.), William Beaumont Army ...
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The Use of Positron Emission Tomography-Computed Tomography Scan in the Evaluation of a Patient with Carney Complex Paul Byron Bandelin, Albert J. Moreno, Homer Jess LeMar, Constantine A. Stratakis, and Thomas George Oliver Department of Medicine (P.B.B.), Nuclear Medicine Service (A.J.M.), and Endocrine Service (H.J.L., T.G.O.), William Beaumont Army Medical Center, El Paso, Texas 79920-5001; and Section on Endocrinology and Genetics (C.A.S.), Program on Developmental Endocrinology & Genetics, National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland 20892

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32-yr-old woman presented with clinical hypercortisolism, including a 30-lb weight gain, central obesity, oligomenorrhea, hirsutism, and hypertension with hypokalemia. The patient reported easy bruisability, and her surgical history included bilateral breast fibrous adenomas within a myxoid stroma. Labs: K⫹ 2.9 mmol/liter, 0800-h cortisol 33 ␮g/dl; 1600-h cortisol 35 ␮g/dl; and 24-h urine-free cortisol 175 ␮g. An adrenal computed tomography (CT) revealed bilateral nodularity; a positron emission tomography (PET)-CT demonstrated F18-fluorodeoxyglucose uptake in both adrenals (Fig. 1 and 2). The patient underwent a two-stage laparoscopic adrenal resection. Pathology revealed primary pigmented nodular adrenocortical disease (PPNAD) (Fig. 3). Carney complex (CNC) is an autosomal dominant multiple neoplasia syndrome associated with hyperfunctioning pituitary, gonads and/or adrenals with hypercortisolism being the most common endocrine finding (1). The diagnosis of Cushing’s syndrome may be elusive: sometimes, abnormal cortisol secretion can only be demonstrated after a dexamethasoneinduced stimulation (2), and adrenal CT can be equivocal (3). PET scan of the adrenals has been used to detect cancers, but PET imaging alone may be ambiguous, and the diagnostic capability is increased with PET-CT, allowing for simultaneous anatomical and functional evaluation of adrenal lesions (4).

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FIG. 1. F18 fluorodeoxyglucose avid adrenal glands, transverse section, adrenal glands at white arrows. Standardized Uptake Values (SUV):Liver-3.0(range 2.75– 3.4), left adrenal-4.45, right adrenal-3.54.

The presence of breast myxomas, PPNAD, and a PRKAR1A mutation confirmed the diagnosis of CNC in our patient; genetic analysis demonstrated a mutation of the PRKAR1A gene (IVS1–2 A⬎G), which has been shown before to cause PPNAD and CNC (5). The patient’s only child, a daughter, carries the same PRKAR1A mutation. This is, to our knowledge, the first report of PET-CT imaging of the adrenal glands in PPNAD. PET-CT imaging may be useful in the evaluation of patients with ACTH-independent hypercortisolism. Apparently, not all F18-fluorodeoxyglucose-avid adrenal glands contain malignancies. Abbreviations: CNC, Carney complex; CT, computed tomography; PET, positron emission tomography; PPNAD, primary pigmented nodular adrenocortical disease.

Printed in U.S.A. Copyright © 2008 by The Endocrine Society doi: 10.1210/jc.2008-0313 Received February 8, 2008. Accepted May 20, 2008.

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J Clin Endocrinol Metab. August 2008, 93(8):2946 –2947

J Clin Endocrinol Metab, August 2008, 93(8):2946 –2947

jcem.endojournals.org

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FIG. 3. Left adrenal gland; the white arrows point to pigmented areas of the nodular cells (lipofuscin granules). Magnification 40⫻.

the Department of the Army, the Department of Defense, or the United States Government. This work was supported by the Intramural Program of the National Institute of Child Health & Human Development (NICHD) and the National Institutes of Health (NIH). Disclosure Statement: The authors have nothing to disclose.

References FIG. 2. F18 fluorodeoxyglucose avid adrenal glands, coronal section, adrenal glands at white arrows. Standardized Uptake Values (SUV):Liver-3.0(range 2.75–3.4), left adrenal-4.45, right adrenal-3.54.

Acknowledgments We would like to thank Dr. David Ranking from WBAMC pathology for specimen preparation and photography. Adress all correspondence and requests for reprints to: Thomas George Oliver, M.D., Endocrine Service, William Beaumont Army Medical Center, 5005 North Piedras Street, El Paso, Texas 79920-5001.H E-mail: [email protected]. The views expressed in this abstract are those of the author(s) and do not reflect the official policy of William Beaumont Army Medical Center,

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 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 3. Courcoutsakis NA, Patronas NJ, Cassarino D, Griffin K, Keil M, Ross JL, Carney JA, Stratakis CA 2004 Hypodense nodularity on computed tomography: novel imaging and pathology of micronodular adrenocortical hyperplasia associated with myelolipomatous changes. J Clin Endocrinol Metab 89:3737–3738 4. Chong S, Lee KS, Kim HY, Kim YK, Kim B-T, Chung MJ, Yi CA, Kwon GY 2006 Integrated PET-CT for the characterization of adrenal gland lesions in cancer patients: diagnostic efficacy and interpretation pitfalls. Radiographics [Erratum (2007) 27:1594] 26:1811–1824 5. Kirschner LS, Sandrini F, Monbo J, Lin JP, Carney JA, Stratakis CA 2000 Genetic heterogeneity and spectrum of mutations of the PRKAR1A gene in patients with the Carney complex. Hum Mol Genet 9:3037–3046