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Endocr Rev. 2014 Apr;35(2):282-326. doi: 10.1210/er.2013-1029. Epub 2013 Dec 20.

Adrenocortical carcinoma.

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  • 1MEND/Division of Metabolism, Endocrinology, and Diabetes (T.E., T.J.G., G.D.H.), Division of Molecular Medicine and Genetics (V.M.R.), Department of Internal Medicine; Departments of Radiation Oncology (A.S., J.S.), Pathology (T.J.G.), and Radiology (A.K., E.M.C.); and Division of Endocrine Surgery (B.S.M.), Section of General Surgery, (A.C.K.), Department of Surgery, University of Michigan Hospital and Health Systems, Ann Arbor, Michigan 48109.


Adrenocortical carcinoma (ACC) is a rare endocrine malignancy, often with an unfavorable prognosis. Here we summarize the knowledge about diagnosis, epidemiology, pathophysiology, and therapy of ACC. Over recent years, multidisciplinary clinics have formed and the first international treatment trials have been conducted. This review focuses on evidence gained from recent basic science and clinical research and provides perspectives from the experience of a large multidisciplinary clinic dedicated to the care of patients with ACC.

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