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A Al-Sharefi Department of Endocrinology, The Royal Victoria Infirmary, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK

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P Perros Department of Endocrinology, The Royal Victoria Infirmary, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK

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R A James Department of Endocrinology, The Royal Victoria Infirmary, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK

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Introduction Phaeochromocytomas/paragangliomas (PHAEO/PG) are chromaffin cell tumours, which can occur sporadically or as part of other hereditary syndromes including multiple endocrine neoplasia 2 (MEN-2), Von Hippel–Lindau syndrome (VHL) and

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Joakim Crona Departments of Surgical Sciences, Medical Sciences, Uppsala University, S-751 85 Uppsala, Sweden

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Alberto Delgado Verdugo Departments of Surgical Sciences, Medical Sciences, Uppsala University, S-751 85 Uppsala, Sweden

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Dan Granberg Departments of Surgical Sciences, Medical Sciences, Uppsala University, S-751 85 Uppsala, Sweden

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Staffan Welin Departments of Surgical Sciences, Medical Sciences, Uppsala University, S-751 85 Uppsala, Sweden

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Peter Stålberg Departments of Surgical Sciences, Medical Sciences, Uppsala University, S-751 85 Uppsala, Sweden

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Per Hellman Departments of Surgical Sciences, Medical Sciences, Uppsala University, S-751 85 Uppsala, Sweden

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Peyman Björklund Departments of Surgical Sciences, Medical Sciences, Uppsala University, S-751 85 Uppsala, Sweden

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Introduction Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare tumours arising from chromaffin cells in adrenal medulla and autonomous ganglia. A majority of these tumours have a low proliferation and seldom metastasize. The understanding

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Sanjeet Kumar Jaiswal Department of Endocrinology, Seth G.S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Vijaya Sarathi Department of Endocrinology, Vydehi Institute of Medical Sciences and Research Center, Bengaluru, Karnataka, India

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Saba Samad Memon Department of Endocrinology, Seth G.S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Robin Garg Department of Endocrinology, Seth G.S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Gaurav Malhotra Department of Nuclear Medicine, Bhabha Atomic Research Centre, Mumbai, Maharashtra, India

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Priyanka Verma Department of Nuclear Medicine, Bhabha Atomic Research Centre, Mumbai, Maharashtra, India

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Ravikumar Shah Department of Endocrinology, Seth G.S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Manjeet Kaur Sehemby Department of Endocrinology, Seth G.S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Virendra A Patil Department of Endocrinology, Seth G.S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Swati Jadhav Department of Endocrinology, Seth G.S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Anurag Ranjan Lila Department of Endocrinology, Seth G.S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Nalini S Shah Department of Endocrinology, Seth G.S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Tushar R Bandgar Department of Endocrinology, Seth G.S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Introduction Pheochromocytomas (PCC)–paragangliomas (PGL) (PPGL) are rare tumors of neural crest origin with malignant potential. The prevalence of metastasis ranges from 2–13% in PCC to 2.4–50% in PGL ( 1 , 2 ). In patients with unresectable

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Natalie Rogowski-Lehmann Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Munich, Germany

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Aikaterini Geroula Institut für Klinische Chemie und Laboratoriumsmedizin, Universitätsklinikum Carl Gustav Carus an der TU Dresden, Dresden, Germany

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Aleksander Prejbisz Department of Hypertension, Institute of Cardiology, Warsaw, Poland

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Henri J L M Timmers Section of Endocrinology, Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, The Netherlands

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Felix Megerle Medizinische Klinik und Poliklinik I des Universitätsklinikums Würzburg, Würzburg, Germany

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Mercedes Robledo Hereditary Endocrine Cancer Group, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro de Investigacion Biomedica en Red de Enfermedades Raras (CIBERER), Madrid, Spain

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Martin Fassnacht Medizinische Klinik und Poliklinik I des Universitätsklinikums Würzburg, Würzburg, Germany

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Stephanie M J Fliedner First Department of Medicine, University Medical Center Schleswig-Holstein, Lübeck, Germany

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Martin Reincke Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Munich, Germany

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Anthony Stell Department of Computing and Information, University of Melbourne, Melbourne Australia

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Andrzej Januszewicz Department of Hypertension, Institute of Cardiology, Warsaw, Poland

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Jacques W M Lenders Section of Endocrinology, Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, The Netherlands
Medizinische Klinik III, Universitätsklinikum Carl Gustav Carus an der Technische Universität Dresden, Dresden, Germany

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Graeme Eisenhofer Institut für Klinische Chemie und Laboratoriumsmedizin, Universitätsklinikum Carl Gustav Carus an der TU Dresden, Dresden, Germany
Medizinische Klinik III, Universitätsklinikum Carl Gustav Carus an der Technische Universität Dresden, Dresden, Germany

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Felix Beuschlein Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Munich, Germany
Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, Universitätsspital Zürich, Zürich, Switzerland

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Introduction Pheochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumors that derive from adrenomedullary tissue (pheochromocytomas) in about 85% of cases and from extra-adrenal chromaffin tissue (paragangliomas) in the remainder

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Xinlei Chen Department of Endocrinology and Metabolism, West China Hospital of Sichuan University, Chengdu, Sichuan, China

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Liru Hu Department of Endocrinology and Metabolism, West China Hospital of Sichuan University, Chengdu, Sichuan, China

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Caojie Liu Department of Endocrinology and Metabolism, West China Hospital of Sichuan University, Chengdu, Sichuan, China

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Guangcheng Ni Department of Endocrinology and Metabolism, West China Hospital of Sichuan University, Chengdu, Sichuan, China

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Yuwei Zhang Department of Endocrinology and Metabolism, West China Hospital of Sichuan University, Chengdu, Sichuan, China

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Introduction Pheochromocytoma and paraganglioma (PPGL) are tumors composed of chromaffin cells, which commonly produce one or more catecholamines: epinephrine, norepinephrine and dopamine. These tumors are rarely silent, and they are

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Jingci Chen Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

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Yan Wu Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

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Pengyan Wang Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

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Huanwen Wu Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

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Anli Tong Department of Endocrinology, Key Laboratory of Endocrinology, National Health Commission of the People’s Republic of China, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

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Xiaoyan Chang Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China

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Introduction Composite pheochromocytoma/paraganglioma (CP) is a rare neoplasm consisting of pheochromocytoma (PCC) or paraganglioma (PGL) combined with developmentally related neurogenic tumor ( 1 ). Its neurogenic component is various

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João Castro-Teles Department of Biomedicine, Faculty of Medicine of the University of Porto, Porto, Portugal

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Bernardo Sousa-Pinto MEDCIDS, Department of Community Medicine, Information and Health Decision Sciences, Faculty of Medicine, University of Porto, Porto, Portugal
CINTESIS, Center for Health Technology and Services Research, University of Porto, Porto, Portugal

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Sandra Rebelo Department of Biomedicine, Faculty of Medicine of the University of Porto, Porto, Portugal
Instituto de Investigação e Inovação em Saúde (i3S), Universidade do Porto, Porto, Portugal
Department of Clinical Pathology, Centro Hospitalar Universitário de São João (CHUSJ), Porto, Portugal

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Duarte Pignatelli Department of Biomedicine, Faculty of Medicine of the University of Porto, Porto, Portugal
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, Centro Hospitalar Universitário de São João (CHUSJ), Porto, Portugal

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neoplasms, such as hemangioblastomas and pheochromocytomas. Pheochromocytomas and paragangliomas (PPGLs), arising either through germline VHL mutations in a syndromic setting or via somatic VHL mutations sporadically, associate with the pseudo

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Debra M Gordon University of the Witwatersrand (WITS) Donald Gordon Medical Centre, Parktown, Johannesburg, South Africa

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Pablo Beckers Department of Human Genetics, Centre Hospitalier Universitaire de Liège, Liège Université, Liège, Belgium

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Emilie Castermans Department of Human Genetics, Centre Hospitalier Universitaire de Liège, Liège Université, Liège, Belgium

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Sebastian J C M M Neggers Department of Endocrinology, Erasmus University Medical Center, Rotterdam, The Netherlands

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Liliya Rostomyan Department of Endocrinology, Centre Hospitalier Universitaire de Liège, Liège Université, Liège, Belgium

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Vincent Bours Department of Human Genetics, Centre Hospitalier Universitaire de Liège, Liège Université, Liège, Belgium

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Patrick Petrossians Department of Endocrinology, Centre Hospitalier Universitaire de Liège, Liège Université, Liège, Belgium

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Vinciane Dideberg Department of Human Genetics, Centre Hospitalier Universitaire de Liège, Liège Université, Liège, Belgium

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Albert Beckers Department of Endocrinology, Centre Hospitalier Universitaire de Liège, Liège Université, Liège, Belgium

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Adrian F Daly Department of Endocrinology, Centre Hospitalier Universitaire de Liège, Liège Université, Liège, Belgium

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particularly true in the case of pheochromocytomas and paragangliomas (PPGL) ( 1 ). These neuroendocrine tumors produce symptoms due to direct tumor effects and the synthesis and release of bioactive amines, neurotransmitters, and hormones; about 20 new

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Gemma White Department of Diabetes and Endocrinology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Anand Velusamy Department of Diabetes and Endocrinology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Samantha Anandappa Department of Diabetes and Endocrinology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Michael Masucci Department of Diabetes and Endocrinology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Louise A Breen Department of Diabetes and Endocrinology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Mamta Joshi Department of Diabetes and Endocrinology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Barbara McGowan Department of Diabetes and Endocrinology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
Faculty of Life Sciences and Medicine, King’s College London, London, UK

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Johnathan G H Hubbard Department of Endocrine Surgery, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Rupert Obholzer Department of Ear, Nose and Throat Surgery, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Dimitra Christodoulou Department of Radiology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Audrey Jacques Department of Radiology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Philip Touska Department of Radiology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Fahim-Ul Hassan Department of Nuclear Medicine, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Louise Izatt Department of Clinical Genetics, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

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Paul V Carroll Department of Diabetes and Endocrinology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
Faculty of Life Sciences and Medicine, King’s College London, London, UK

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Introduction Phaeochromocytomas and paragangliomas (PPGLs) are neuroendocrine tumours arising from neural crest tissue. Phaeochromocytoma (PCC) arises from the adrenal medulla and paragangliomas (PGLs) from autonomic ganglia, usually arising

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Yiqiang Huang Department of Urology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, People’s Republic of China

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Lin-ang Wang Department of Urology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, People’s Republic of China

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Qiubo Xie Department of Urology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, People’s Republic of China

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Jian Pang Department of Urology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, People’s Republic of China

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Luofu Wang Department of Urology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, People’s Republic of China

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Yuting Yi Geneplus-Beijing Institute, Beijing, People’s Republic of China

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Jun Zhang Department of Urology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, People’s Republic of China

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Yao Zhang Department of Urology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, People’s Republic of China

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Rongrong Chen Geneplus-Beijing Institute, Beijing, People’s Republic of China

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Weihua Lan Department of Urology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, People’s Republic of China

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Dianzheng Zhang Department of Bio-Medical Sciences, Philadelphia College of Osteopathic Medicine, Philadelphia, Pennsylvania, USA

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Jun Jiang Department of Urology, Institute of Surgery Research, Daping Hospital, Third Military Medical University, Chongqing, People’s Republic of China

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Introduction Pheochromocytomas/paragangliomas (PCC/PGLs) are tumors, arose from neural crest-derived chromaffin cells, produce and secrete catecholamines ( 1 , 2 , 3 ). PCCs are tumors of the adrenal medulla and PGLs originate from

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