Search for other papers by Jülide Durmuşoğlu in
Google Scholar
PubMed
Search for other papers by Henri J L M Timmers in
Google Scholar
PubMed
Search for other papers by Pepijn van Houten in
Google Scholar
PubMed
Search for other papers by Johan F Langenhuijsen in
Google Scholar
PubMed
Search for other papers by Ad R M M Hermus in
Google Scholar
PubMed
Search for other papers by Annenienke C van de Ven in
Google Scholar
PubMed
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
Search for other papers by Wenhao Lin in
Google Scholar
PubMed
Search for other papers by Jun Dai in
Google Scholar
PubMed
Search for other papers by Jialing Xie in
Google Scholar
PubMed
Search for other papers by Jiacheng Liu in
Google Scholar
PubMed
Search for other papers by Fukang Sun in
Google Scholar
PubMed
Search for other papers by Xin Huang in
Google Scholar
PubMed
Search for other papers by Wei He in
Google Scholar
PubMed
Search for other papers by Chen Fang in
Google Scholar
PubMed
Search for other papers by Juping Zhao in
Google Scholar
PubMed
Search for other papers by Danfeng Xu in
Google Scholar
PubMed
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
Search for other papers by Emily Warmington in
Google Scholar
PubMed
Search for other papers by Gabrielle Smith in
Google Scholar
PubMed
Search for other papers by Vasileios Chortis in
Google Scholar
PubMed
Department of Neurosurgery, Technical University Munich (TMU), Munich, Germany
Search for other papers by Raimunde Liang in
Google Scholar
PubMed
Search for other papers by Juliane Lippert in
Google Scholar
PubMed
Search for other papers by Sonja Steinhauer in
Google Scholar
PubMed
Search for other papers by Laura-Sophie Landwehr in
Google Scholar
PubMed
Medizinische Klinik Und Poliklinik III, University Hospital Carl Gustav Carus, Dresden, Germany
Search for other papers by Constanze Hantel in
Google Scholar
PubMed
Search for other papers by Katja Kiseljak-Vassiliades in
Google Scholar
PubMed
Search for other papers by Margaret E Wierman in
Google Scholar
PubMed
Search for other papers by Barbara Altieri in
Google Scholar
PubMed
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
Search for other papers by Paul A Foster in
Google Scholar
PubMed
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
Search for other papers by Cristina L Ronchi in
Google Scholar
PubMed
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
Search for other papers by Jung Soo Lim in
Google Scholar
PubMed
Search for other papers by Seung-Eun Lee in
Google Scholar
PubMed
Search for other papers by Jung Hee Kim in
Google Scholar
PubMed
Search for other papers by Jae Hyeon Kim in
Google Scholar
PubMed
Search for other papers by The Korean Adrenal Gland and Endocrine Hypertension Study Group, Korean Endocrine Society in
Google Scholar
PubMed
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
Department of Endocrinology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France
Search for other papers by Anne Jouinot in
Google Scholar
PubMed
Search for other papers by Juliane Lippert in
Google Scholar
PubMed
Comprehensive Cancer Center Mainfranken, University of Wuerzburg, Wuerzburg, Germany
Search for other papers by Martin Fassnacht in
Google Scholar
PubMed
Search for other papers by Bruno de La Villeon in
Google Scholar
PubMed
Search for other papers by Amandine Septier in
Google Scholar
PubMed
Search for other papers by Mario Neou in
Google Scholar
PubMed
Search for other papers by Karine Perlemoine in
Google Scholar
PubMed
Search for other papers by Silke Appenzeller in
Google Scholar
PubMed
Department of Pathology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France
Search for other papers by Mathilde Sibony in
Google Scholar
PubMed
Department of Digestive and Endocrine Surgery, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France
Search for other papers by Sébastien Gaujoux in
Google Scholar
PubMed
Department of Digestive and Endocrine Surgery, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France
Search for other papers by Bertrand Dousset in
Google Scholar
PubMed
Department of Endocrinology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France
Search for other papers by Rossella Libe in
Google Scholar
PubMed
Department of Endocrinology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France
Search for other papers by Lionel Groussin in
Google Scholar
PubMed
Institute of Metabolism and System Research, University of Birmingham, Birmingham, UK
Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
Search for other papers by Cristina L Ronchi in
Google Scholar
PubMed
Department of Endocrinology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France
Search for other papers by Guillaume Assié in
Google Scholar
PubMed
Department of Endocrinology, Assistance Publique Hôpitaux de Paris, Hôpital Cochin, Paris, France
Search for other papers by Jérôme Bertherat in
Google Scholar
PubMed
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
Search for other papers by Magdalena Lech in
Google Scholar
PubMed
Search for other papers by Ruvini Ranasinghe in
Google Scholar
PubMed
Faculty of Life Sciences and Medicine, School of Life Course Sciences, King’s College London, London, UK
Search for other papers by Royce P Vincent in
Google Scholar
PubMed
Search for other papers by David R Taylor in
Google Scholar
PubMed
Search for other papers by Lea Ghataore in
Google Scholar
PubMed
Search for other papers by James Luxton in
Google Scholar
PubMed
Quebec Heart and Lung Institute, Laval University, Quebec, Canada
Search for other papers by Fannie Lajeunesse-Trempe in
Google Scholar
PubMed
Search for other papers by Pia Roser in
Google Scholar
PubMed
Search for other papers by Eftychia E Drakou in
Google Scholar
PubMed
Search for other papers by Ling Ling Chuah in
Google Scholar
PubMed
Barts and the London School of Medicine, Centre for Endocrinology, William Harvey Institute, London, UK
Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK
Search for other papers by Ashley B Grossman in
Google Scholar
PubMed
Search for other papers by Simon J B Aylwin in
Google Scholar
PubMed
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
Search for other papers by Georgios K Dimitriadis in
Google Scholar
PubMed
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
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
Search for other papers by Sofia S Pereira in
Google Scholar
PubMed
Search for other papers by Mariana P Monteiro in
Google Scholar
PubMed
Search for other papers by Sonir R Antonini in
Google Scholar
PubMed
Institute of Molecular Pathology and Immunology of the University of Porto (IPATIMUP), Porto, Portugal
Department of Endocrinology, Hospital S. João, Porto, Portugal
Search for other papers by Duarte Pignatelli in
Google Scholar
PubMed
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
Search for other papers by Weixi Wang in
Google Scholar
PubMed
Search for other papers by Rulai Han in
Google Scholar
PubMed
Search for other papers by Lei Ye in
Google Scholar
PubMed
Search for other papers by Jing Xie in
Google Scholar
PubMed
Search for other papers by Bei Tao in
Google Scholar
PubMed
Search for other papers by Fukang Sun in
Google Scholar
PubMed
Search for other papers by Ran Zhuo in
Google Scholar
PubMed
Search for other papers by Xi Chen in
Google Scholar
PubMed
Search for other papers by Xiaxing Deng in
Google Scholar
PubMed
Search for other papers by Cong Ye in
Google Scholar
PubMed
Search for other papers by Hongyan Zhao in
Google Scholar
PubMed
Search for other papers by Shu Wang in
Google Scholar
PubMed
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
Institut Cochin, INSERM U1016, CNRS UMR8104, Université Paris Descartes, Sorbonne Paris Cité, Paris, France
Search for other papers by Anne Jouinot in
Google Scholar
PubMed
Search for other papers by Bernard Royer in
Google Scholar
PubMed
Search for other papers by Etienne Chatelut in
Google Scholar
PubMed
Search for other papers by Sotheara Moeung in
Google Scholar
PubMed
Department of Endocrinology, Center for Rare Adrenal Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France
Search for other papers by Guillaume Assié in
Google Scholar
PubMed
Pharmacokinetics and Pharmacochemistry Unit, Cochin Hospital, Paris Descartes University, AP-HP, Paris, France
Search for other papers by Audrey Thomas-Schoemann in
Google Scholar
PubMed
Department of Endocrinology, Center for Rare Adrenal Diseases, Hôpital Cochin, Assistance Publique Hôpitaux de Paris, Paris, France
Search for other papers by Jérôme Bertherat in
Google Scholar
PubMed
Search for other papers by François Goldwasser in
Google Scholar
PubMed
UMR8638 CNRS, Pharmacy UFR, University of Paris Descartes, PRES sorbonne Paris Cité, Paris, France
Search for other papers by Benoit Blanchet in
Google Scholar
PubMed
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
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
Search for other papers by Sofia S Pereira in
Google Scholar
PubMed
Search for other papers by Tiago Morais in
Google Scholar
PubMed
Search for other papers by Madalena M Costa in
Google Scholar
PubMed
Search for other papers by Mariana P Monteiro in
Google Scholar
PubMed
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
Search for other papers by Duarte Pignatelli in
Google Scholar
PubMed
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