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Current Issues in the Diagnosis and Management of Adrenocortical Carcinomas.


Endotext [Internet]. South Dartmouth (MA):, Inc.; 2000-.
2016 Mar 6.

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Professor of Medicine, University of California, San Francisco, CA
Chief of Medicine at the University of Washington Medical Center and Professor and Vice Chair of the Department of Medicine, University of Washington
Pediatric Endocrinologist and Associate Research Physician in the Skeletal Diseases and Mineral Homeostasis Section, National Institute of Dental and Craniofacial Research, National Institutes of Health
Professor of Pediatrics and Endocrinology, Division of Endocrinology, Metabolism and Diabetes, First Department of Pediatrics, National and Kapodistrian University of Athens Medical School, "Aghia Sophia" Children's Hospital, Athens, Greece
Associate Professor of Medicine, Division of Endocrinology, Diabetes, and Metabolism, Ohio State University
Professor of Endocrinology and Director of the Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, UK
Distinguished Professor of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA; Associate Chief, Endocrinology and Diabetes Division and Director, Endocrine Clinic, West Los Angeles VA Medical Center, Los Angeles, CA
Professor of General Medicine-Endocrinology, First Department of Propaedeutic Internal Medicine, Laiko University Hospital, Athens, Greece
Head of the Medicover MVZ Oldenburg; affiliated with the Carl von Ossietzky University and the Technical University of Dresden
Professor of Medicine and Chief of the Division of Endocrinology, Diabetology and Metabolism, University of Lausanne, Switzerland
Professor of Endocrinology and Metabolism, Centre Lead for Endocrinology and Deputy Institute Director, William Harvey Research Institute, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, London, England
Director of Clinical Research, Hudson Institute of Medical Research; Consultant Endocrinologist, Monash Medical Centre, Melbourne, Australia
Dammert Professor of Gerontology and Director, Division of Geriatric Medicine and Director of the Division of Endocrinology, Saint Louis University Medical Center
Professor of Pediatrics, Professor of Genetics and Genomic Sciences, and Chief of the Adrenal Steroid Disorders Program, Icahn School of Medicine, Mount Sinai School of Medicine, New York, NY
Associate Professor of Medicine and Epidemiology, University of Colorado Anschutz Medical Campus
Professor of Medicine, Knight Cardiovascular Institute and the Division of Endocrinology, and Associate Director, Bob and Charlee Moore Institute for Nutrition and Wellness, Oregon Health and Science University, Portland, OR
Professor and Chair, Department of Obstetrics and Gynecology, Western Michigan University Homer Stryker M.D. School of Medicine, Kalamazoo, MI
Director of the Endocrine/Bone Disease Program, John Wayne Cancer Institute at Saint John’s Health Center, Santa Monica, CA; Clinical Professor of Medicine, UCLA School of Medicine, Los Angeles, CA
Director of the Diabetes Care Center and Associate Professor of Medicine, University of Washington Medical Center, Seattle, WA
Murray Waitzer Endowed Chair for Diabetes Research, Professor of Medicine/Pathology/Neurobiology, Director of Research and Neuroendocrine Unit Division of Endocrine and Metabolic Disorders, Eastern Virginia Medical School, Norfolk, VA
Endowed Chair, Cardiovascular Health and Risk Prevention, Pediatric Endocrinology and Diabetes, Cook Children's Medical Center, Fort Worth, TX
Department of Biological Chemistry, Medical School, National University of Athens, 75 Mikras Asias Str., Goudi, Athens, 11521, Greece
MD, Department of Pathophysiology, Sector of Endocrinology,National and Kapodistrian University of Athens, Greece, Medical School, Mikras Asias 75, 11527 Goudi,
MD,Consultant Surgeon, Athens General Hospital 'G.Gennimatas', 10 K. Ourani str, Athens, 15237, Greece,
Dr. Gregory Kaltsas MD FRCP (Lon) is a Professor in General Medicine and Endocrinology at the National and Kapodistrian University of Athens, Greece. He was trained in General Medicine in Athens, Greece and London UK and in Endocrinology at the Middlesex and St Bartholomew’s Hospital, London, UK. He developed a particular interest in Neuroendocrinology (pituitary and neuroendocrine tumors) and adrenal physiology and diseases. Upon returning to Greece he established a neuroendocrine network and he is currently running the European Neuroendocrine Tumor Society (ENETS) Center of Excellence at Laiko Hospital in Athens, Greece. He has served as a member of the advisory board of ENETS and of the Executive Committee of the European Neuroendocrine Association (ENEA) and he has been elected in the Executive Committee of the International Society of Endocrinology. He has published more than 300 original papers, reviews and chapters and serves on editorial boards and associate editor in several endocrine journals.
Division of Endocrinology, Metabolism and Diabetes (N.C.N., G.P.C., E.C.), First Department of Pediatrics, National and Kapodistrian University of Athens Medical School, ‘Aghia Sophia’ Children's Hospital, Athens, 11527, Greece; Division of Endocrinology and Metabolism (N.C.N., G.P.C., E.C.), Center of Clinical, Experimental Surgery and Translational Research, Biomedical Research Foundation of the Academy of Athens, Athens, 11527, Greece


Adrenocortical carcinoma (ACC) is a rare and aggressive malignant tumor, and management requires a multidisciplinary approach. Although several molecular and immunohistochemical markers have recently evolved as predictors of aggressiveness, none of them has been established for a particular therapeutic strategy. Ki-67 expression has been found to be the single most prognostic factor for recurrence of ACCs. Surgery remains the ‘gold standard’ in resectable tumors. The high recurrence rate and poor outcome of ACCs have prompted use of adjuvant therapy following surgical resection. In unresectable tumors or in metastatic disease, other therapeutic modalities are considered. Mitotane is the standard medical treatment in cases of advanced or aggressive tumors. It requires close monitoring because it is associated with adverse side-effects that may compromise its use. EDP chemotherapy along with mitotane is the recommended therapeutic regimen in metastatic adrenocortical carcinomas not amenable to surgery, but has no significant benefit in the overall survival. New targeted therapies based on tumor biology are being developed in order to improve therapeutic efficacy. Prospective clinical trials should be initiated in low and high risk patients to evaluate current and evolving therapies.

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