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Adrenal Cortical Adenoma: The Fourth Component of the Carney Triad and an Association With Subclinical Cushing Syndrome

Carney, J. Aidan MD, PhD, FRCP, FRCPI*; Stratakis, Constantine A. MD, D(med)Sci; Young, William F. Jr MD, MSc

American Journal of Surgical Pathology: August 2013 - Volume 37 - Issue 8 - p 1140–1149
doi: 10.1097/PAS.0b013e318285f6a2
Original Articles

The Carney triad is the combination of gastric stromal sarcoma, pulmonary chondroma, and extra-adrenal paraganglioma. Herein, we describe the clinical, imaging, pathologic, and follow-up findings from 14 patients for a fourth component of the syndrome, adrenal adenoma. The adrenal neoplasm was asymptomatic and usually a late finding. Results of adrenocortical function tests were normal. Computed tomography revealed low-density adrenal masses that were consistent with adenomas. Bilateral lesions were present in 4 patients. In 13 of the 14 patients who underwent surgery, resected adrenal glands and biopsy specimens featured 1 or more circumscribed, yellow tumors, up to 3.5 cm in diameter, composed of well-differentiated polygonal cells with clear vacuolated cytoplasm and a smaller component of eosinophilic cells. The extratumoral cortex had combinations of normal histologic features, discrete clear cell micronodules, zonal clear cell hypertrophy, and marked atrophy. The lesion in the 14th patient was different, grossly and microscopically resembling the usual sporadic cortisol-secreting adenoma. After the tumor was excised, the patient required glucocorticoid support. None of the tumors recurred or metastasized. Fourteen additional patients had unilateral or bilateral adrenal tumors consistent with adenomas detected by imaging studies.

*Department of Laboratory Medicine and Pathology

Division of Endocrinology, Diabetes, Metabolism and Nutrition, Mayo Clinic, Rochester, MN

Section on Endocrinology & Genetics, Program on Developmental Endocrinology and Genetics, NICHD, NIH, Bethesda, MD

Conflicts of Interest and Source of Funding: C.A.S. was supported by Intramural Program, NICHD, NIH, project: HD008920-01. The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: J. Aidan Carney, MD, PhD, FRCP, FRCPI, Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (e-mail: carney.aidan@mayo.edu).

© 2013 by Lippincott Williams & Wilkins.