The Journal of Clinical Endocrinology & Metabolism Vol. 93, No. 8 2946-2947
Copyright © 2008 by The Endocrine Society
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
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: Thomas.george.oliver{at}us.army.mil.
A 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 µg/dl; 1600-h cortisol 35 µg/dl; and 24-h urine-free cortisol 175 µg. 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).
View larger version (53K):
[in this window]
[in a new window]
|
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.
|
|
View larger version (51K):
[in this window]
[in a new window]
|
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.
|
|
View larger version (159K):
[in this window]
[in a new window]
|
FIG. 3. Left adrenal gland; the white arrows point to pigmented areas of the nodular cells (lipofuscin granules). Magnification 40x.
|
|
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 dexamethasone-induced 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).
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.
 |
Acknowledgments
|
|---|
We would like to thank Dr. David Ranking from WBAMC pathology for specimen preparation and photography.
|
Footnotes
|
|---|
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, 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.
Abbreviations: CNC, Carney complex; CT, computed tomography; PET, positron emission tomography; PPNAD, primary pigmented nodular adrenocortical disease.
Received February 8, 2008.
Accepted May 20, 2008.
|
References
|
|---|
- 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]
- 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]
- 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[Free Full Text]
- 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
- 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[Abstract/Free Full Text]
Top
References
