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Journal of Oncology
Volume 2012 (2012), Article ID 234726, 11 pages
Review Article

Adrenocortical Carcinoma: Current Therapeutic State-of-the-Art

Department of Surgery, Yale Endocrine Neoplasia Laboratory, Yale School of Medicine, 333 Cedar Street, TMP202, Box 208062, New Haven, CT 06520, USA

Received 26 May 2012; Revised 13 August 2012; Accepted 27 August 2012

Academic Editor: Bruce H. R. Wolffenbuttel

Copyright © 2012 Amir H. Lebastchi et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Adrenocortical carcinoma (ACC) is a rare, aggressive malignancy that generally conveys a poor prognosis. Currently, surgical resection is considered the lone curative treatment modality. In addition, the low prevalence of ACC has limited effective clinical trial design to develop evidence-based approaches to ACC therapy. The proper role of radio- and chemotherapy treatment for ACC is still being defined. Similarly, the molecular pathogenesis of ACC remains to be fully characterized. Despite these challenges, progress has been made in several areas. After years of refinement, an internationally accepted staging system has been defined. International collaborations have facilitated increasingly robust clinical trials, especially regarding agent choice and patient selection for chemotherapeutics. Genetic array data and molecular profiling have identified new potential targets for rational drug design as well as potential tumor markers and predictors of therapeutic response. However, these advances have not yet been translated into a large outcomes benefit for ACC patients. In this paper, we summarize established therapy for ACC and highlight recent findings in the field that are impacting clinical practice.