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Deborah Cosentini Medical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, ASST Spedali Civili, Brescia, Italy

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Salvatore Grisanti Medical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, ASST Spedali Civili, Brescia, Italy

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Alberto Dalla Volta Medical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, ASST Spedali Civili, Brescia, Italy

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Marta Laganà Medical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, ASST Spedali Civili, Brescia, Italy

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Chiara Fiorentini Section of Pharmacology, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy

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Paola Perotti Internal Medicine, Department of Clinical and Biological Sciences, San Luigi Hospital, University of Turin, Orbassano, Italy

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Sandra Sigala Section of Pharmacology, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy

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Alfredo Berruti Medical Oncology Unit, Department of Medical and Surgical Specialties, Radiological Sciences, and Public Health, University of Brescia, ASST Spedali Civili, Brescia, Italy

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Editorial Adrenocortical carcinoma (ACC) is a rare endocrine neoplasia characterized by an overall dismal prognosis and its clinical manifestations are the consequence of either steroid excess or tumor mass progression ( 1 ). Surgery is the

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Eric Seidel Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Nephrology and Medical Intensive Care, BCRT – Berlin Institute of Health Center for Regenerative Therapies, Berlin, Germany
Berlin Institute of Health (BIH), Berlin, Germany
Department of Nephrology, School of Medicine, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany

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Gudrun Walenda Department of Nephrology, School of Medicine, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany

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Clemens Messerschmidt Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany
Core Unit Bioinformatics, Berlin Institute of Health, Berlin, Germany

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Benedikt Obermayer Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany
Core Unit Bioinformatics, Berlin Institute of Health, Berlin, Germany

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Mirko Peitzsch Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany

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Paal Wallace Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany

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Rohini Bahethi Department of Nephrology, School of Medicine, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany

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Taekyeong Yoo Department of Biomedical Sciences, Seoul National University College of Medicine, Seoul, Republic of Korea

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Murim Choi Department of Biomedical Sciences, Seoul National University College of Medicine, Seoul, Republic of Korea

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Petra Schrade Charité – Universitaetsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Institut für Vegetative Anatomie, Berlin, Germany

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Sebastian Bachmann Charité – Universitaetsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Institut für Vegetative Anatomie, Berlin, Germany

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Gerhard Liebisch Institute of Clinical Chemistry and Laboratory Medicine, Regensburg University Hospital, Regensburg, Germany

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Graeme Eisenhofer Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany

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Dieter Beule Core Unit Bioinformatics, Berlin Institute of Health, Berlin, Germany
Max Delbrück Center for Molecular Medicine in the Helmholtz Association, Berlin, Germany

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Ute I Scholl Charité – Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Department of Nephrology and Medical Intensive Care, BCRT – Berlin Institute of Health Center for Regenerative Therapies, Berlin, Germany
Berlin Institute of Health (BIH), Berlin, Germany
Department of Nephrology, School of Medicine, Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany

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, Berlin) for bioinformatics support. References 1 Fassnacht M Kroiss M Allolio B . Update in adrenocortical carcinoma . Journal of Clinical Endocrinology and Metabolism 2013 4551 – 4564 . ( https://doi.org/10.1210/jc.2013-3020 ) 2

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Maria Cristina De Martino Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands
Dipartimento di Medicina Clinica e Chirurgia, Università Federico II, Naples, Italy

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Richard A Feelders Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands

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Claudia Pivonello Dipartimento di Medicina Clinica e Chirurgia, Università Federico II, Naples, Italy

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Chiara Simeoli Dipartimento di Medicina Clinica e Chirurgia, Università Federico II, Naples, Italy

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Fortuna Papa Dipartimento di Medicina Clinica e Chirurgia, Università Federico II, Naples, Italy

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Annamaria Colao Dipartimento di Medicina Clinica e Chirurgia, Università Federico II, Naples, Italy

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Rosario Pivonello Dipartimento di Medicina Clinica e Chirurgia, Università Federico II, Naples, Italy

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Leo J Hofland Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands

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Introduction Adrenocortical carcinomas (ACCs) are rare tumors with scant treatment options for which new treatments are required ( 1 , 2 , 3 ). The limited efficacy of conventional antineoplastic treatment in ACCs increases the need for

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Manjunath Goroshi Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India
Jawaharlal Nehru Medical College, Belagavi, India

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Swati S Jadhav Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Vijaya Sarathi Narayana Medical College and Hospital, Nellore, Andhra Pradesh, India

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Anurag R Lila Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Virendra A Patil Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Ravikumar Shah Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Priya Hira Department of Radiology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Rajaram Sharma Department of Radiology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Shettepppa Goroshi Department of Radiology, Sri Nijalingappa Medical College Bagalkot, Navanagar, Karnataka, India

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Gwendolyn Fernandes Department of Pathology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Amey Rojekar Department of Pathology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Abhay Dalvi Departments of General Surgery, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Ganesh Bakshi Department of Uro-Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India

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Gagan Prakash Department of Uro-Oncology, Tata Memorial Hospital, Mumbai, Maharashtra, India

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Nalini S Shah Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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Tushar R Bandgar Department of Endocrinology, Seth GS Medical College and KEM Hospital, Parel, Mumbai, India

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adrenocortical carcinoma (ACC, 8%), phaeochromocytoma (PCC, 7%) and adrenal metastasis (AM, 5%) are other less common masses ( 1 ). Most crucial step in management of an adrenal incidentaloma is to differentiate a benign ‘leave-alone’ lesion, from the one that

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L Ghataore Departments of, Clinical Biochemistry, Medicine, Department of Endocrinology and Internal Medicine, King's College Hospital, London SE5 9RS, UK

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I Chakraborti Departments of, Clinical Biochemistry, Medicine, Department of Endocrinology and Internal Medicine, King's College Hospital, London SE5 9RS, UK

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S J Aylwin Departments of, Clinical Biochemistry, Medicine, Department of Endocrinology and Internal Medicine, King's College Hospital, London SE5 9RS, UK

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K-M Schulte Departments of, Clinical Biochemistry, Medicine, Department of Endocrinology and Internal Medicine, King's College Hospital, London SE5 9RS, UK

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D Dworakowska Departments of, Clinical Biochemistry, Medicine, Department of Endocrinology and Internal Medicine, King's College Hospital, London SE5 9RS, UK
Departments of, Clinical Biochemistry, Medicine, Department of Endocrinology and Internal Medicine, King's College Hospital, London SE5 9RS, UK

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P Coskeran Departments of, Clinical Biochemistry, Medicine, Department of Endocrinology and Internal Medicine, King's College Hospital, London SE5 9RS, UK

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N F Taylor Departments of, Clinical Biochemistry, Medicine, Department of Endocrinology and Internal Medicine, King's College Hospital, London SE5 9RS, UK

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Introduction Mitotane (o,p'-DDD) plays an important role in adjuvant therapy of adrenocortical carcinoma (ACC) and in advanced stage disease (1) . This application was first reported in 1959 by Bergenstal et al . (2) , but mitotane has only been

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Milène Tetsi Nomigni INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France
INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France

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Sophie Ouzounian INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France

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Alice Benoit INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France

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Jacqueline Vadrot INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France

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Frédérique Tissier INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France

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Sylvie Renouf INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France
INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France

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Hervé Lefebvre INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France
INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France
INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France

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Sophie Christin-Maitre INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France
INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France

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Estelle Louiset INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France
INSERM, University of Rouen, Department of Endocrinology, Departments of Endocrinology, Pathology, Department of Pathology, Department of Endocrinology, INSERM, U982, Laboratory of Neuronal and Neuroendocrine Differentiation and Communication, Institute for Research and Innovation in Biomedicine, Mont‐Saint‐Aignan, France

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androgen and cortisol oversecretion is highly suggestive of adrenocortical carcinoma (12) . In very few cases, androgen-secreting adrenal lesions have been identified as bilateral macronodular hyperplasia (13, 14) , adenoma (15) , primary pigmented

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Emmanuelle Motte UFR des Sciences de la Santé, Simone Veil, Université Versailles St-Quentin en Yvelines, Montigny le Bretonneux, France
Assistance Publique Hôpitaux de Paris (APHP), Department of Endocrinology and Diabetes for Children, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France

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Anya Rothenbuhler Assistance Publique Hôpitaux de Paris (APHP), Department of Endocrinology and Diabetes for Children, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France
APHP, Plateforme d’Expertise Maladies Rares Paris Sud, Bicêtre Paris Sud Hospital, Le Kremlin Bicêtre, France

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Stephan Gaillard Department of Neurosurgery, Foch Hospital, Suresnes, France

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Najiba Lahlou APHP, Department of Hormonal Biology, Cochin Hospital, Paris, France

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Cécile Teinturier Assistance Publique Hôpitaux de Paris (APHP), Department of Endocrinology and Diabetes for Children, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France
APHP, Plateforme d’Expertise Maladies Rares Paris Sud, Bicêtre Paris Sud Hospital, Le Kremlin Bicêtre, France

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Régis Coutant Department of Pediatric Endocrinology, Angers University Hospital, Angers, France

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Agnès Linglart Assistance Publique Hôpitaux de Paris (APHP), Department of Endocrinology and Diabetes for Children, Bicêtre Paris-Sud, Le Kremlin Bicêtre, France
APHP, Plateforme d’Expertise Maladies Rares Paris Sud, Bicêtre Paris Sud Hospital, Le Kremlin Bicêtre, France

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cytotoxic effect on the zona reticularis of adrenal glands ( 17 , 18 , 19 ). Its therapeutic use has been validated in adults with metastatic adrenocortical carcinoma, with a target concentration of 14–20 mg/L. At lower concentrations, mitotane reduces

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Gamze Akkuş Division of Endocrinology, Internal Medicine and Endocrinology, Cukurova University Medical Faculty, Department of Internal Medicine, Adana, Turkey

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Isa Burak Güney Nuclear Medicine Department, Cukurova University Medical Faculty, Adana, Turkey

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Fesih Ok Urology Department, Cukurova University Medical Faculty, Adana, Turkey

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Mehtap Evran Division of Endocrinology, Internal Medicine and Endocrinology, Cukurova University Medical Faculty, Department of Internal Medicine, Adana, Turkey

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Volkan Izol Urology Department, Cukurova University Medical Faculty, Adana, Turkey

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Şeyda Erdoğan Pathology Department, Cukurova University Medical Faculty, Adana, Turkey

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Yıldırım Bayazıt Urology Department, Cukurova University Medical Faculty, Adana, Turkey

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Murat Sert Division of Endocrinology, Internal Medicine and Endocrinology, Cukurova University Medical Faculty, Department of Internal Medicine, Adana, Turkey

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Tamer Tetiker Division of Endocrinology, Internal Medicine and Endocrinology, Cukurova University Medical Faculty, Department of Internal Medicine, Adana, Turkey

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adrenocortical adenoma (Weiss criteria <4) and two as adrenocortical carcinoma. One of the two patients with adrenocortical carcinoma was diagnosed with pheochromocytoma (Pass criteria >4, SUV max 8.2). The other patient had a non-secretory adrenocortical

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Wafaa M Rashed Research Department, Children’s Cancer Hospital Egypt (CCHE-57357), Cairo, Egypt
Armed Forces College of Medicine, Cairo, Egypt

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Anas Saad Faculty of Medicine, Damascus University, Damascus, Syria

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Muneer Al-Husseini Faculty of Medicine, Ain Shams University, Cairo, Egypt

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Ahmed Mahmoud Galal Armed Forces College of Medicine, Cairo, Egypt

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Assem Mohamed Ismael Armed Forces College of Medicine, Cairo, Egypt

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Ahmed M Al-Tayep Armed Forces College of Medicine, Cairo, Egypt

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Ayman El Shafie Armed Forces College of Medicine, Cairo, Egypt

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Mahmoud Ahmed Ali Armed Forces College of Medicine, Cairo, Egypt

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Ahmad Samir Alfaar Charité – Universitätsmedizin Berlin, Campus Virchow Klinikum, Ophthalmology Department, Berlin, Germany

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adrenal gland tumors and BRCA1 or BRCA2 mutations. These genetic associations should be investigated in large longitudinal study of primary breast cancer and adrenal gland tumor as a second malignancy. The incidence of adrenocortical carcinomas (ACCs) as

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Boju Pan Department of Pathology, Molecular Pathology Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Anqi Wang Clinical Biobank, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Junyi Pang Department of Pathology, Molecular Pathology Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Yuhan Zhang Department of Pathology, Molecular Pathology Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Ming Cui Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Jian Sun Department of Pathology, Molecular Pathology Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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Zhiyong Liang Department of Pathology, Molecular Pathology Research Center, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China

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parathyroid adenoma and carcinoma. Numerous studies have revealed PD-L1 expression in endocrine tumors, including pituitary tumors ( 11 ), papillary thyroid carcinomas ( 12 , 13 ), adrenocortical carcinomas ( 14 ), pheochromocytomas ( 15 ) and

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