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WenQi Yuan Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China
Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China

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WeiQinq Wang Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China
Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China

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Bin Cui Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China
Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China

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TingWei Su Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China

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Yan Ge Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China
Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China

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Lei Jiang Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China

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WeiWei Zhou Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China

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Guang Ning Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China
Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China
Department of Endocrine and Metabolic Diseases, Laboratory of Endocrine and Metabolic Diseases, Division of Endocrine and Metabolic Diseases, School of Medicine, Shanghai Clinical Center for Endocrine and Metabolic Diseases, Ruijin Hospital, Shanghai JiaoTong University, 197 Ruijin Er Lu, Shanghai 200025, People's Republic of China

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sympathetic nervous system. Both the clinical and biochemical features of pheochromocytomas are mostly the result of the overproduction of catecholamines. In sporadic pheochromocytomas and paragangliomas, ∼20% of them are associated with germline mutations

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Birke Bausch
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Ulrich Wellner 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Dirk Bausch 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Francesca Schiavi 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Marta Barontini 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Gabriela Sanso 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Martin K Walz 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Mariola Peczkowska 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Georges Weryha 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Patrizia Dall'Igna 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Giovanni Cecchetto 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Gianni Bisogno 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Lars C Moeller 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Detlef Bockenhauer 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Attila Patocs 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Karoly Rácz 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Dmitry Zabolotnyi 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Svetlana Yaremchuk 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Iveta Dzivite-Krisane 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Frederic Castinetti 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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David Taieb 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Angelica Malinoc 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Ernst von Dobschuetz 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Jochen Roessler 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Kurt W Schmid 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Giuseppe Opocher 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Charis Eng 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Hartmut P H Neumann 2nd Department of Medicine, Department of Surgery, Department of Medicine, Center for Endocrinological Investigations (CEDIE), Department of Surgery, Department of Hypertension, Department of Endocrinology, Division of Pediatric Surgery, Pediatric Oncology, Department of Endocrinology, Department of Pediatrics, 2nd Department of Internal Medicine, Institute of Otorhinolaryngology, Department of Endocrinology, Department of Endocrinology, Department of Nuclear Medicine, Section for Preventive Medicine, Department of Visceral Surgery, Department of Pediatrics, Department of Pathology, Genomic Medicine Institute, University of Freiburg, Freiburg, Germany

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Introduction Pheochromocytomas and paragangliomas are tumors of the overall paraganglial system, mainly the adrenal glands, the retroperitoneum, the pelvis, the thorax, and the skullbase and neck regions ( Neumann 2008 ). Such paraganglial tumors

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Lucia Martiniova Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA
Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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Shiromi M Perera Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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Frederieke M Brouwers Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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Salvatore Alesci Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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Mones Abu-Asab Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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Amanda F Marvelle Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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Dale O Kiesewetter Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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David Thomasson Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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John C Morris Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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Richard Kvetnansky Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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Arthur S Tischler Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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James C Reynolds Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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Antonio Tito Fojo Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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Karel Pacak Section on Medical Neuroendocrinology, Clinical Neuroendocrinology Branch, Laboratory of Pathology, Intramural Science PRGMS, Laboratory of Diagnostic Radiology, Metabolism Branch, Institute of Experimental Endocrinology, Department of Pathology, Nuclear Medicine Department, Medical Oncology Branch, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, Room 1E-3140, 10 Center Drive MSC-1109, Bethesda, Maryland 20892-1109, USA

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Introduction Up to 36% of patients diagnosed with pheochromocytoma or paraganglioma will develop metastatic disease and have a 5-year survival of ∼50% ( O'Riordain et al . 1996 , Mundschenk & Lehnert 1998 , John et al . 1999 , Plouin et al

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Martin Ullrich Department of Radiopharmaceutical and Chemical Biology, Helmholtz-Zentrum Dresden-Rossendorf, Institute of Radiopharmaceutical Cancer Research, Dresden, Germany

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Josephine Liers Department of Radiopharmaceutical and Chemical Biology, Helmholtz-Zentrum Dresden-Rossendorf, Institute of Radiopharmaceutical Cancer Research, Dresden, Germany
Technische Universität Dresden, School of Medicine, Faculty of Medicine Carl Gustav Carus, Dresden, Germany

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

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Anja Feldmann Department of Radioimmunology, Helmholtz-Zentrum Dresden-Rossendorf, Institute of Radiopharmaceutical Cancer Research, Dresden, Germany

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Ralf Bergmann Department of Radiopharmaceutical and Chemical Biology, Helmholtz-Zentrum Dresden-Rossendorf, Institute of Radiopharmaceutical Cancer Research, Dresden, Germany

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Ulrich Sommer Technische Universität Dresden, University Hospital Carl Gustav Carus, Institute of Pathology, Dresden, Germany

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Susan Richter Technische Universität Dresden, School of Medicine, Faculty of Medicine Carl Gustav Carus, Dresden, Germany
Technische Universität Dresden, University Hospital Carl Gustav Carus, Institute of Clinical Chemistry and Laboratory Medicine, Dresden, Germany

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Stefan R Bornstein Technische Universität Dresden, School of Medicine, Faculty of Medicine Carl Gustav Carus, Dresden, Germany
Department of Internal Medicine III, Technische Universität Dresden, University Hospital Carl Gustav Carus, Dresden, Germany

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Michael Bachmann Technische Universität Dresden, School of Medicine, Faculty of Medicine Carl Gustav Carus, Dresden, Germany
Department of Radioimmunology, Helmholtz-Zentrum Dresden-Rossendorf, Institute of Radiopharmaceutical Cancer Research, Dresden, Germany
Technische Universität Dresden, University Hospital Carl Gustav Carus, Universitäts Krebs Centrum (UCC), Tumorimmunology, Dresden, Germany
Technische Universität Dresden, National Center for Tumor Diseases (NCT), Dresden, Germany

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

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Christian G Ziegler Department of Internal Medicine III, Technische Universität Dresden, University Hospital Carl Gustav Carus, Dresden, Germany

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Jens Pietzsch Department of Radiopharmaceutical and Chemical Biology, Helmholtz-Zentrum Dresden-Rossendorf, Institute of Radiopharmaceutical Cancer Research, Dresden, Germany
Technische Universität Dresden, School of Science, Faculty of Chemistry and Food Chemistry, Dresden, Germany

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Introduction Adrenal pheochromocytomas and extra adrenal paragangliomas (PPGLs) are rare catecholamine-producing tumors of chromaffin cell origin ( Lenders et al. 2005 , Harari & Inabnet 2011 , Jemal et al. 2011 ). In contrast to most

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Umasuthan Srirangalingam
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Bernard Khoo
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Lisa Walker Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Fiona MacDonald Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Robert H Skelly Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Emad George Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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David Spooner Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Linda B Johnston
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John P Monson
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Ashley B Grossman
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W M Drake
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Scott A Akker
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Patrick J Pollard Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Nick Plowman Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Norbert Avril Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Daniel M Berney Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Jacky M Burrin Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Rodney H Reznek Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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V K Ajith Kumar Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Eamonn R Maher Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Shern L Chew
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Introduction Recent studies have shown that ∼25% of apparently sporadic phaeochromocytomas and paragangliomas are due to germ line mutations in one of several familial syndrome genes, including von Hippel-Lindau ( VHL ), RET , succinate

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Jenny Welander Department of Clinical and Experimental Medicine, Department of Oncology-Pathology, Department of Surgery, Department of Clinical Science, Department of Molecular Medicine and Surgery, Department of Surgery, Faculty of Health Sciences, Linköping University, Linköping SE-58185, Sweden

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Adam Andreasson Department of Clinical and Experimental Medicine, Department of Oncology-Pathology, Department of Surgery, Department of Clinical Science, Department of Molecular Medicine and Surgery, Department of Surgery, Faculty of Health Sciences, Linköping University, Linköping SE-58185, Sweden

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Michael Brauckhoff Department of Clinical and Experimental Medicine, Department of Oncology-Pathology, Department of Surgery, Department of Clinical Science, Department of Molecular Medicine and Surgery, Department of Surgery, Faculty of Health Sciences, Linköping University, Linköping SE-58185, Sweden
Department of Clinical and Experimental Medicine, Department of Oncology-Pathology, Department of Surgery, Department of Clinical Science, Department of Molecular Medicine and Surgery, Department of Surgery, Faculty of Health Sciences, Linköping University, Linköping SE-58185, Sweden

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Martin Bäckdahl Department of Clinical and Experimental Medicine, Department of Oncology-Pathology, Department of Surgery, Department of Clinical Science, Department of Molecular Medicine and Surgery, Department of Surgery, Faculty of Health Sciences, Linköping University, Linköping SE-58185, Sweden

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Catharina Larsson Department of Clinical and Experimental Medicine, Department of Oncology-Pathology, Department of Surgery, Department of Clinical Science, Department of Molecular Medicine and Surgery, Department of Surgery, Faculty of Health Sciences, Linköping University, Linköping SE-58185, Sweden

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Oliver Gimm Department of Clinical and Experimental Medicine, Department of Oncology-Pathology, Department of Surgery, Department of Clinical Science, Department of Molecular Medicine and Surgery, Department of Surgery, Faculty of Health Sciences, Linköping University, Linköping SE-58185, Sweden
Department of Clinical and Experimental Medicine, Department of Oncology-Pathology, Department of Surgery, Department of Clinical Science, Department of Molecular Medicine and Surgery, Department of Surgery, Faculty of Health Sciences, Linköping University, Linköping SE-58185, Sweden

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Peter Söderkvist Department of Clinical and Experimental Medicine, Department of Oncology-Pathology, Department of Surgery, Department of Clinical Science, Department of Molecular Medicine and Surgery, Department of Surgery, Faculty of Health Sciences, Linköping University, Linköping SE-58185, Sweden

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Introduction Pheochromocytomas and abdominal paragangliomas are catecholamine-producing tumors derived from neuroendocrine chromaffin cells in the adrenal medulla or the extra-adrenal paraganglia. The tumors can be a manifestation of different

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Paraskevi Xekouki Section on Endocrinology and Genetics (SEGEN), Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), Inter-Institute Pediatric Endocrinology Program, National Institutes of Health (NIH), Building 10, CRC, Room 1-3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Constantine A Stratakis Section on Endocrinology and Genetics (SEGEN), Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), Inter-Institute Pediatric Endocrinology Program, National Institutes of Health (NIH), Building 10, CRC, Room 1-3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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for the formation of multiple and possibly coexisting parasympathetic and sympathetic paragangliomas (PGLs) and/or pheochromocytomas (PHEOs) ( Baysal et al . 2000 , Astuti et al . 2001 ) made obsolete ( Dluhy 2002 ) at least one part of the axiom

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Asterios Karagiannis
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Dimitri P Mikhailidis Division of Endocrinology, Department of Clinical Biochemistry,, Second Propedeutic Department of Internal Medicine, Medical School, Hippokration Hospital, Aristotle University of Thessaloniki, Thessaloniki 54642, Greece

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Vasilios G Athyros
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Faidon Harsoulis
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intraadrenal paraganglioma (PGL). Adrenal and extraadrenal PGLs produce significant amounts of catecholamines and give rise to the well-known clinical picture of PHEOs. In contrast, the parasympathetic PGLs (mainly in head and neck) rarely produce significant

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Eva Szarek Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Evan R Ball Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Alessio Imperiale Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA
Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Maria Tsokos Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Fabio R Faucz Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Alessio Giubellino Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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François-Marie Moussallieh Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA
Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Izzie-Jacques Namer Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA
Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Mones S Abu-Asab Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Karel Pacak Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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David Taïeb Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA
Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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J Aidan Carney Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Constantine A Stratakis Section on Endocrinology and Genetics (SEGEN), Department of Biophysics and Nuclear Medicine, Faculty of Medicine, Laboratory of Pathology, Section on Medical Neuroendocrinology (SMN), Section of Immunopathology and Laboratory of Immunology, Department of Nuclear Medicine, Institut Paoli‐Calmettes, Emeritus Staff Center, Program on Developmental Endocrinology and Genetics (PDEGEN), Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1‐3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA

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Introduction Carney triad (CTr) is a syndrome that describes the association of paragangliomas (PGLs) with gastrointestinal (GI) stromal tumors (GISTs) and pulmonary chondromas; other lesions, including pheochromocytomas, esophageal leiomyomas, and

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Sophie Moog Université de Paris Cité, PARCC INSERM UMR970, Paris, France

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Betty Salgues PARCC INSERM UMR970, Paris, France
Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Radiologie, Paris, France

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Yasmine Braik-Djellas Université de Paris Cité, PARCC INSERM UMR970, Paris, France

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Thomas Viel Université de Paris Cité, PARCC INSERM UMR970, Paris, France
Sorbonne Université, Service de Médecine Nucléaire, Assistance Publique-Hôpitaux de Paris, Hôpital Pitié Salpêtrière, Paris, France

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Daniel Balvay Université de Paris Cité, PARCC INSERM UMR970, Paris, France
Sorbonne Université, Service de Médecine Nucléaire, Assistance Publique-Hôpitaux de Paris, Hôpital Pitié Salpêtrière, Paris, France

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Gwennhael Autret Université de Paris Cité, PARCC INSERM UMR970, Paris, France
Sorbonne Université, Service de Médecine Nucléaire, Assistance Publique-Hôpitaux de Paris, Hôpital Pitié Salpêtrière, Paris, France

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Estelle Robidel Université de Paris Cité, PARCC INSERM UMR970, Paris, France

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Anne-Paule Gimenez-Roqueplo Université de Paris Cité, PARCC INSERM UMR970, Paris, France
Plateforme d’Imageries du Vivant, Université de Paris Cité, Faculté de Médecine, Paris, France

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Bertrand Tavitian Université de Paris Cité, PARCC INSERM UMR970, Paris, France
Sorbonne Université, Service de Médecine Nucléaire, Assistance Publique-Hôpitaux de Paris, Hôpital Pitié Salpêtrière, Paris, France
Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Génétique, Paris, France

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Charlotte Lussey-Lepoutre PARCC INSERM UMR970, Paris, France
Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Radiologie, Paris, France

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Judith Favier Université de Paris Cité, PARCC INSERM UMR970, Paris, France

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Introduction Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors that arise in chromaffin cells of the adrenal medulla or in sympathetic and parasympathetic ganglia, respectively. Around 40% of PPGL patients carry a

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