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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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, and SDHx respectively ( Welander et al . 2011 , Dahia 2014 ). In recent years, additional susceptibility genes for pheochromocytoma and paraganglioma have been discovered, including TMEM127 ( Qin et al . 2010 ), MAX ( Comino-Mendez et al

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Thomas G Papathomas Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Lindsey Oudijk Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Ellen C Zwarthoff Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Edward Post Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Floor A Duijkers Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Max M van Noesel Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Leo J Hofland Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Patrick J Pollard Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Eamonn R Maher Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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David F Restuccia Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Richard A Feelders Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Gaston J H Franssen Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Henri J Timmers Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Stefan Sleijfer Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Wouter W de Herder Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Ronald R de Krijger Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands
Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Winand N M Dinjens Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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Esther Korpershoek Department of Pathology Josephine Nefkens Institute, Erasmus MC, Rotterdam, The Netherlands

Department of Pediatric Oncology–Hematology Erasmus MC–Sophia Children's Hospital, Rotterdam, The Netherlands

Sector of Endocrinology Department of Internal Medicine, Erasmus MC, Rotterdam, The Netherlands

Cancer Biology and Metabolism Group Institute of Genetics and Molecular Medicine, Edinburgh Cancer Research UK Centre, University of Edinburgh, Edinburgh, UK

Department of Medical Genetics University of Cambridge, Cambridge, UK

Department of Surgery Erasmus MC, Rotterdam, The Netherlands

Division of Endocrinology Department of Medicine, Radboud University Nijmegen Medical Center, Nijmegen, The Netherlands

Department of Medical Oncology Erasmus MC, Rotterdam, The Netherlands

Department of Pathology Reinier de Graaf Hospital, Delft, The Netherlands

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peripheral neuroblastic tumors (Erasmus MC, Rotterdam, The Netherlands: 15 NBLs, eight ganglioneuroblastomas, and ten ganglioneuromas). Tumors with mutations in the SDH-x genes, such as SDHA , SDHB , SDHC , SDHD , and SDHAF2 , display loss of

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Karel Pacak Section of Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA

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Mark Kidd WREN Laboratories, Branford, Connecticut, USA

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Leah Meuter Section of Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA

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Irvin M Modlin Gastroenterological and Endoscopic Surgery, Yale University School of Medicine, New Haven, Connecticut, USA

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). Nineteen of the 58 cases (33%) were mutation-negative. Twenty-nine (50%) exhibited mutations in the SDHx cluster (predominantly SDHB : n   = 25). The remaining samples exhibited individual mutations in PHEO/PGL-associated genes. Cluster 1-associated

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Bora E Baysal
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Eamonn R Maher Department of Pathology, Department of Medical Genetics, Roswell Park Cancer Institute, Buffalo, New York 14263, USA

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defined association. Carney–Stratakis syndrome is characterized by the association of GIST with paraganglioma and, in most cases is caused by mutations in SDHX genes ( McWhinney et al . 2007 , Janeway et al . 2011 ). Germline SDHX mutation may also be

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Trisha Dwight Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, St Leonards, New South Wales, Australia
University of Sydney, Sydney, New South Wales, Australia

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Edward Kim Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, St Leonards, New South Wales, Australia
University of Sydney, Sydney, New South Wales, Australia

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Karine Bastard Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, St Leonards, New South Wales, Australia
University of Sydney, Sydney, New South Wales, Australia

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Diana E Benn Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, St Leonards, New South Wales, Australia
University of Sydney, Sydney, New South Wales, Australia

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

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

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Massimo Mannelli Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

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Elena Rapizzi Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

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

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Mariola Pęczkowska Department of Hypertension, National Institute of Cardiology, Warsaw, Poland

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Karel Pacak National Institutes of Health, Bethesda, Maryland, USA

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Roderick Clifton-Bligh Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, St Leonards, New South Wales, Australia
University of Sydney, Sydney, New South Wales, Australia
Department of Endocrinology, Royal North Shore Hospital, St Leonards, New South Wales, Australia

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) and Polish ( n = 70) subsets; Table 2 and Supplementary Tables 1, 2, see section on supplementary materials given at the end of this article). As shown in Table 2, an additional 15 subjects (of whom five also had germline SDHx mutations) were

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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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germline mutation in one of the 20 PPGL susceptibility genes identified so far. Mutations in SDHx ( SDHA , SDHB , SDHC , SDHD , and SDHAF2 ) genes are the most frequently involved, causing approximately half of the genetically determined cases

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Yuji Ikeda Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA

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Kazuma Kiyotani Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA

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Poh Yin Yew Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA

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Taigo Kato Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA

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Kenji Tamura Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA

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Kai Lee Yap Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA

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Sarah M Nielsen Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA

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Jessica L Mester Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA

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Charis Eng Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA
Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA

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Yusuke Nakamura Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA

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Raymon H Grogan Section of Hematology/Oncology, Genomic Medicine Institute, Department of Genetics and Genome Sciences, Endocrine Surgery Research Program, Department of Medicine, The University of Chicago, Chicago, Illinois 60637, USA

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mutations in other genes, such as succinate dehydrogenase genes ( SDHx ) (8%), PIK3CA (8.8%), AKT1 (2.2%), and SEC23B (4%) are also known as CS/CLS susceptibility genes ( Ni et al . 2008 , Ngeow et al . 2011 , Orloff et al . 2013 ). CS/CLS is one

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Jenny Welander Department of Clinical and Experimental Medicine, Department of Surgery, Faculty of Health Sciences, Linköping University, 58185 Linköping, Sweden

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Peter Söderkvist Department of Clinical and Experimental Medicine, Department of Surgery, Faculty of Health Sciences, Linköping University, 58185 Linköping, Sweden

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Oliver Gimm Department of Clinical and Experimental Medicine, Department of Surgery, Faculty of Health Sciences, Linköping University, 58185 Linköping, Sweden
Department of Clinical and Experimental Medicine, Department of Surgery, Faculty of Health Sciences, Linköping University, 58185 Linköping, Sweden

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that 95% of the patients had PCC and 6% had PGL, all of which were sympathetic. Fourteen percent of the patients displayed bilateral PCC, 9% developed malignant disease, and the mean age at presentation was 42 years. SDHx Genes and protein functions SDH

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Fabio R Faucz Section on Endocrinology & Genetics, Program on Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America

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Anelia D Horvath Department of Pharmacology and Physiology, School of Medicine and Health Sciences, The George Washington University, Washington, District of Columbia, United States of America

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Guillaume Assié Université Paris Cité, CNRS UMR8104, INSERM U1016, Institut Cochin, Paris, France
Endocrine Department, Center for Rare Adrenal Diseases, AP-HP, Hôpital Cochin, Paris, France

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Madson Q Almeida Section on Endocrinology & Genetics, Program on Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America
Adrenal Unit, Laboratory of Molecular and Cellular Endocrinology LIM/25, Division of Endocrinology and Metabolism, University of Sao Paulo Medical School, São Paulo, Brasil

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Eva Szarek Section on Endocrinology & Genetics, Program on Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America

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Sosipatros Boikos Section on Endocrinology & Genetics, Program on Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America

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Anna Angelousi Section on Endocrinology & Genetics, Program on Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America

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Isaac Levy Section on Endocrinology & Genetics, Program on Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America

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Andrea G Maria Section on Endocrinology & Genetics, Program on Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America

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Ajay Chitnis Laboratory of Molecular Genetics, Section on Neural Developmental Dynamics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America

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Cristina R Antonescu Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York, United States of America

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Rainer Claus Hematology and Oncology, Medical Faculty, University of Augsburg, Augsburg, Germany

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Jérôme Bertherat Université Paris Cité, CNRS UMR8104, INSERM U1016, Institut Cochin, Paris, France
Endocrine Department, Center for Rare Adrenal Diseases, AP-HP, Hôpital Cochin, Paris, France

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Christoph Plass Division of Cancer Epigenomics, German Cancer Research Center, Heidelberg, Germany

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Charis Eng Genomic Medicine Institute, Lerner Research Institute, and Taussig Cancer Institute, Cleveland Clinic, Cleveland, Ohio, United States of America

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Constantine A Stratakis Section on Endocrinology & Genetics, Program on Developmental Endocrinology & Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, United States of America
Human Genetics & Precision Medicine, IMBB, Foundation for Research & Technology Hellas, Heraklion, Crete, Greece
Research Institute, ELPEN, Pikermi, Athens, Greece

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definition, we included only SDHB-negative tumors by IHC in the study because we were looking for genes mutated in SDHB-negative tumors that were negative for SDHx pathogenic variants. Also to be included in the study, all patients were SDHA , SDHB , SDHC

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Ernst von Dobschuetz Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Helena Leijon Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Camilla Schalin-Jäntti Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Francesca Schiavi Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Michael Brauckhoff Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Mariola Peczkowska Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Giovanna Spiazzi Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Serena Demattè Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Maria Enrica Cecchini Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Paola Sartorato Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Jolanta Krajewska Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Kornelia Hasse-Lazar Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Katarzyna Roszkowska-Purska Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Elisa Taschin Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Angelica Malinoc Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Lars A Akslen Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Johanna Arola Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Dariusz Lange Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Ambrogio Fassina Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Gianmaria Pennelli Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Mattia Barbareschi Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Jutta Luettges Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Aleksander Prejbisz Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Andrzej Januszewicz Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Tim Strate Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Birke Bausch Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Frederic Castinetti Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Barbara Jarzab Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Giuseppe Opocher Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Charis Eng Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Hartmut P H Neumann Section of Endocrine Surgery, Department of Pathology, Division of Endocrinology, Familial Cancer Clinic and Onco‐Endocrinology, Department of Endocrine Surgery, Department of Hypertension, Endocrinology, Units of Internal Medicine, Department of Internal Medicine, Department of Nuclear Medicine and Endocrine Oncology, Department of Pathology and Laboratory Diagnostics, Section for Preventive Medicine, Section for Pathology, Department of Tumor Pathology, Surgical Pathology and Cytopathology Unit, Department of Pathology, Department of Pathology, Department of Gastroenterology, Department of Endocrinology, Genomic Medicine Institute, Department of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, St. Adolf Stift, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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SDHx genes ( Zantour et al . 2004 , Boedeker et al . 2009 ). Heritable non- SDHx paraganglioma can also occur in tumor syndromes mainly associated with pheochromocytomas such as von Hippel–Lindau disease (associated with mutations of the VHL gene

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