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Jülide Durmuşoğlu Division of Endocrinology, Department of Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands

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Henri J L M Timmers Division of Endocrinology, Department of Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands

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Pepijn van Houten Division of Endocrinology, Department of Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands

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Johan F Langenhuijsen Department of Urology, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands

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Ad R M M Hermus Division of Endocrinology, Department of Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands

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Annenienke C van de Ven Division of Endocrinology, Department of Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, the Netherlands

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Introduction Adrenocortical carcinoma (ACC) is a rare and usually aggressive malignancy of the adrenal cortex. It has an annual incidence of approximately one case per million individuals ( 1 , 2 ). The pillar of treatment for ACC is complete

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Wenhao Lin Department of Urology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

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Jun Dai Department of Urology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

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Jialing Xie Department of Pathology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

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Jiacheng Liu Department of Urology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

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Fukang Sun Department of Urology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

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Xin Huang Department of Urology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

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Wei He Department of Urology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

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Chen Fang Department of Urology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

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Juping Zhao Department of Urology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

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Danfeng Xu Department of Urology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China

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Introduction Adrenocortical carcinoma (ACC) is a rare and aggressive cancer that affects both children and adults, having an overall incidence of 1–2 cases/million per year. In most cases, cancer presents with steroid hormone excess ( 1

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Emily Warmington Institute of Metabolism and System Research, University of Birmingham, Birmingham, UK

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Gabrielle Smith Institute of Metabolism and System Research, University of Birmingham, Birmingham, UK

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Vasileios Chortis Institute of Metabolism and System Research, University of Birmingham, Birmingham, UK

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Raimunde Liang Division of Endocrinology and Diabetes, University Hospital of Wuerzburg, Wuerzburg, Germany
Department of Neurosurgery, Technical University Munich (TMU), Munich, Germany

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Juliane Lippert Division of Endocrinology and Diabetes, University Hospital of Wuerzburg, Wuerzburg, Germany

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Sonja Steinhauer Division of Endocrinology and Diabetes, University Hospital of Wuerzburg, Wuerzburg, Germany

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Laura-Sophie Landwehr Division of Endocrinology and Diabetes, University Hospital of Wuerzburg, Wuerzburg, Germany

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Constanze Hantel Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH), Zurich, Switzerland
Medizinische Klinik Und Poliklinik III, University Hospital Carl Gustav Carus, Dresden, Germany

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Katja Kiseljak-Vassiliades Division of Endocrinology Metabolism and Diabetes, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA

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Margaret E Wierman Division of Endocrinology Metabolism and Diabetes, University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA

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Barbara Altieri Division of Endocrinology and Diabetes, University Hospital of Wuerzburg, Wuerzburg, Germany

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Paul A Foster Institute of Metabolism and System Research, University of Birmingham, Birmingham, UK
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK

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Cristina L Ronchi Institute of Metabolism and System Research, University of Birmingham, Birmingham, UK
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK

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Introduction Adrenocortical carcinoma (ACC) is a rare yet highly aggressive endocrine malignancy with generally poor prognosis ( 1 ). Treatment options for ACC are scarce, with the only potential curative therapy being complete resection ( 2

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Jung Soo Lim Department of Internal Medicine, Institute of Evidence-Based Medicine, Wonju Severance Christian Hospital, Yonsei University Wonju College of Medicine, Wonju, Gangwon-do, South Korea

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Seung-Eun Lee Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Gangnam-gu, Seoul, South Korea

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Jung Hee Kim Department of Internal Medicine, Seoul National University College of Medicine, Jongno-gu, Seoul, South Korea

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Jae Hyeon Kim Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Gangnam-gu, Seoul, South Korea

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The Korean Adrenal Gland and Endocrine Hypertension Study Group, Korean Endocrine Society
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Introduction Adrenocortical carcinoma (ACC) is a rare disease with an estimated annual incidence of 0.7–2.0 per million population ( 1 , 2 ). The prevalence of ACC varies depending on the circumstances under which patient data are collected

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Anne Jouinot Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France
Department of Endocrinology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France

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Juliane Lippert Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Wuerzburg, Wuerzburg, Germany

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Martin Fassnacht Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Wuerzburg, Wuerzburg, Germany
Comprehensive Cancer Center Mainfranken, University of Wuerzburg, Wuerzburg, Germany

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Bruno de La Villeon Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France

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Amandine Septier Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France

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Mario Neou Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France

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Karine Perlemoine Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France

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Silke Appenzeller Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Wuerzburg, Wuerzburg, Germany

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Mathilde Sibony Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France
Department of Pathology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France

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Sébastien Gaujoux Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France
Department of Digestive and Endocrine Surgery, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France

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Bertrand Dousset Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France
Department of Digestive and Endocrine Surgery, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France

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Rossella Libe Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France
Department of Endocrinology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France

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Lionel Groussin Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France
Department of Endocrinology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France

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Cristina L Ronchi Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Wuerzburg, Wuerzburg, Germany
Institute of Metabolism and System Research, University of Birmingham, Birmingham, UK
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK

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Guillaume Assié Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France
Department of Endocrinology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France

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Jérôme Bertherat Université de Paris, Institut Cochin, INSERM U1016, CNRS UMR8104, Paris, France
Department of Endocrinology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France

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Introduction Adrenocortical carcinoma is a rare cancer with poor but heterogeneous prognosis. The 5-year overall survival of ACC patients remains below 40% in most series ( 1 ). The main prognostic factors used in clinical practice at

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Magdalena Lech Faculty of Life Sciences and Medicine, School of Life Course Sciences, King’s College London, London, UK

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Ruvini Ranasinghe Department of Clinical Biochemistry, King’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK

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Royce P Vincent Department of Clinical Biochemistry, King’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK
Faculty of Life Sciences and Medicine, School of Life Course Sciences, King’s College London, London, UK

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David R Taylor Department of Clinical Biochemistry, King’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK

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Lea Ghataore Department of Clinical Biochemistry, King’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK

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James Luxton Department of Clinical Biochemistry, King’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK

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Fannie Lajeunesse-Trempe Department of Endocrinology ASO/EASO COM, King's College Hospital NHS Foundation Trust, Denmark Hill, London, UK
Quebec Heart and Lung Institute, Laval University, Quebec, Canada

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Pia Roser Department of Endocrinology and Diabetes, University Medical Centre Hamburg Eppendorf, Hamburg, Germany

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Eftychia E Drakou Department of Clinical Oncology, Guy's Cancer Centre - Guy's and St Thomas' NHS Foundation Trust, Great Maze Pond, London, UK

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Ling Ling Chuah Department of Endocrinology ASO/EASO COM, King's College Hospital NHS Foundation Trust, Denmark Hill, London, UK

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Ashley B Grossman Green Templeton College, University of Oxford, Oxford, UK
Barts and the London School of Medicine, Centre for Endocrinology, William Harvey Institute, London, UK
Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK

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Simon J B Aylwin Department of Endocrinology ASO/EASO COM, King's College Hospital NHS Foundation Trust, Denmark Hill, London, UK

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Georgios K Dimitriadis Department of Endocrinology ASO/EASO COM, King's College Hospital NHS Foundation Trust, Denmark Hill, London, UK
Obesity, Type 2 Diabetes and Immunometabolism Research Group, School of Cardiovascular and Metabolic Medicine & Sciences, Faculty of Life Course Sciences, King’s College London, London, UK
Division of Reproductive Health, Warwick Medical School, University of Warwick, Coventry, UK

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Introduction Adrenocortical carcinoma (ACC) is a rare malignancy of the adrenal cortex with an annual incidence of between 0.5 and 2.0 cases per million ( 1 ). Whilst most cases of ACC are sporadic, typically occurring in the fifth and sixth

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Sofia S Pereira Instituto de Investigação e Inovação em Saúde (I3S), Universidade do Porto, Porto, Portugal
Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Porto, Portugal
Endocrine, Cardiovascular & Metabolic Research, Department of Anatomy, Multidisciplinary Unit for Biomedical Research (UMIB), Instituto de Ciências Biomédicas Abel Salazar, University of Porto (ICBAS/UP), Porto, Portugal

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Mariana P Monteiro Endocrine, Cardiovascular & Metabolic Research, Department of Anatomy, Multidisciplinary Unit for Biomedical Research (UMIB), Instituto de Ciências Biomédicas Abel Salazar, University of Porto (ICBAS/UP), Porto, Portugal

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Sonir R Antonini Department of Pediatrics, Ribeirao Preto Medical School, University of Sao Paulo, Ribeirao Preto, Brazil

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Duarte Pignatelli Instituto de Investigação e Inovação em Saúde (I3S), Universidade do Porto, Porto, Portugal
Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Porto, Portugal
Department of Endocrinology, Hospital S. João, Porto, Portugal

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performed for unrelated clinical reasons ( 1 , 2 ). On the other side, adrenocortical carcinomas (ACC) are rare but usually have an aggressive behavior and a poor prognosis ( 1 , 3 , 4 , 5 , 6 ). According to the ENSAT classification, the 5-year disease

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Weixi Wang Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-Institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China

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Rulai Han Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-Institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China

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Lei Ye Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-Institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China

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Jing Xie Department of Pathology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, Shanghai, China

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Bei Tao Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-Institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China

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Fukang Sun Department of Urology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, Shanghai, China

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Ran Zhuo Department of Urology, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, Shanghai, China

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Xi Chen Department of General Surgery, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China

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Xiaxing Deng Pancreatic Disease Centre, Ruijin Hospital, Shanghai Jiao Tong University, School of Medicine, Shanghai, China

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Cong Ye Department of Thoracic Surgery, Shanghai Pulmonary Hospital, Tongji University, Shanghai, China

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Hongyan Zhao Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-Institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China

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Shu Wang Shanghai Key Laboratory for Endocrine Tumors, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Shanghai Institute of Endocrine and Metabolic Diseases and Shanghai E-Institute for Endocrinology, Ruijin Hospital, Shanghai Jiaotong University, School of Medicine, Shanghai, China

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cases and the majority is adrenocortical adenomas or hyperplasia. A small fraction of MEN1 patients developed adrenocortical carcinoma (ACC) ( 1 , 2 , 3 , 4 , 5 , 6 , 7 , 8 , 9 ). In fact, ACC in general is a rare malignancy with high mortality

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Anne Jouinot Department of Medical Oncology, Cochin Hospital, Paris Descartes University, CARPEM, AP-HP, Paris, France
Institut Cochin, INSERM U1016, CNRS UMR8104, Université Paris Descartes, Sorbonne Paris Cité, Paris, France

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Bernard Royer CHU Besançon, Clinical Pharmacology and Toxicology Dpt, Besançon cedex, France

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Etienne Chatelut Institut Claudius-Regaud, Université de Toulouse, INSERM, Centre de Recherches en Cancérologie de Toulouse, Toulouse, France

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Sotheara Moeung Institut Claudius-Regaud, Université de Toulouse, INSERM, Centre de Recherches en Cancérologie de Toulouse, Toulouse, France

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Guillaume Assié Institut Cochin, INSERM U1016, CNRS UMR8104, Université Paris Descartes, Sorbonne Paris Cité, Paris, France
Department of Endocrinology, Center for Rare Adrenal Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France

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Audrey Thomas-Schoemann Department of Pharmacy, Cochin Hospital, Paris Descartes University, AP-HP, Paris, France
Pharmacokinetics and Pharmacochemistry Unit, Cochin Hospital, Paris Descartes University, AP-HP, Paris, France

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Jérôme Bertherat Institut Cochin, INSERM U1016, CNRS UMR8104, Université Paris Descartes, Sorbonne Paris Cité, Paris, France
Department of Endocrinology, Center for Rare Adrenal Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France

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François Goldwasser Department of Medical Oncology, Cochin Hospital, Paris Descartes University, CARPEM, AP-HP, Paris, France

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Benoit Blanchet Pharmacokinetics and Pharmacochemistry Unit, Cochin Hospital, Paris Descartes University, AP-HP, Paris, France
UMR8638 CNRS, Pharmacy UFR, University of Paris Descartes, PRES sorbonne Paris Cité, Paris, France

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Introduction Adrenocortical carcinoma (ACC) is a rare malignancy (1 per million each year) with a poor prognosis ( 1 ). Even after complete surgery, up to 70% of patients will develop recurrence ( 1 ). Medical treatments show limited efficacy

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Sofia S Pereira Department of Anatomy and UMIB (Unit for Multidisciplinary Biomedical Research) of ICBAS, Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Department of Endocrinology, University of Porto, Porto, 4050-313, Portugal
Department of Anatomy and UMIB (Unit for Multidisciplinary Biomedical Research) of ICBAS, Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Department of Endocrinology, University of Porto, Porto, 4050-313, Portugal

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Tiago Morais Department of Anatomy and UMIB (Unit for Multidisciplinary Biomedical Research) of ICBAS, Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Department of Endocrinology, University of Porto, Porto, 4050-313, Portugal

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Madalena M Costa Department of Anatomy and UMIB (Unit for Multidisciplinary Biomedical Research) of ICBAS, Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Department of Endocrinology, University of Porto, Porto, 4050-313, Portugal

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Mariana P Monteiro Department of Anatomy and UMIB (Unit for Multidisciplinary Biomedical Research) of ICBAS, Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Department of Endocrinology, University of Porto, Porto, 4050-313, Portugal

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Duarte Pignatelli Department of Anatomy and UMIB (Unit for Multidisciplinary Biomedical Research) of ICBAS, Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Department of Endocrinology, University of Porto, Porto, 4050-313, Portugal
Department of Anatomy and UMIB (Unit for Multidisciplinary Biomedical Research) of ICBAS, Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Department of Endocrinology, University of Porto, Porto, 4050-313, Portugal

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prevalence of more than 4% in the adult population; in contrast, adrenocortical carcinomas (ACCs) are rare, having an incidence of between 0.5 and 2 per million, and are generally highly aggressive, with a poor prognosis that is expressed by a 5-year survival

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