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James F Powers Department of Pathology and Laboratory Medicine, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts, USA

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Brent Cochran Department of Developmental, Molecular and Chemical Biology

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James D Baleja Department of Developmental, Molecular and Chemical Biology

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Hadley D Sikes Department of Chemical Engineering, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA

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Andrew D Pattison Department of Clinical Pathology, University of Melbourne, Melbourne, Victoria, Australia

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Xue Zhang Department of Developmental, Molecular and Chemical Biology

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Inna Lomakin Department of Pathology and Laboratory Medicine, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts, USA

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Annette Shepard-Barry Department of Pathology and Laboratory Medicine, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts, USA

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

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Sun Jin Moon Department of Chemical Engineering, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA

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Troy F Langford Department of Chemical Engineering, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA

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Kassi Taylor Stein Department of Chemical Engineering, Massachusetts Institute of Technology, Cambridge, Massachusetts, USA

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Richard W Tothill Department of Clinical Pathology, University of Melbourne, Melbourne, Victoria, Australia
Peter MacCallum Cancer Centre, Melbourne, Victoria, Australia

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Yingbin Ouyang Cyagen US Inc, Santa Clara, California, USA

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Arthur S Tischler Department of Pathology and Laboratory Medicine, Tufts Medical Center, Tufts University School of Medicine, Boston, Massachusetts, USA

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, Castro-Vega et al. 2015 , Favier et al. 2015 , Fishbein et al. 2017 , Fishbein & Wilkerson 2018 ). Tumors with gene mutations of SDH subunits fall into cluster 1, which is characterized by pseudohypoxic signaling and metabolism ( Eisenhofer et

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-19-0474
Volume/Issue:
Volume 27: Issue 6
Open access
Katharina Wang Department of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany

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Ina Schütze Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH), Zurich, Switzerland

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Sebastian Gulde Institute for Diabetes and Cancer (IDC), Helmholtz Center Munich, German Research Center for Environmental Health, Neuherberg, Germany

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Nicole Bechmann Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
Department of Internal Medicine III, University Hospital Carl Gustav Carus Dresden, 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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Jana Helm Department of Internal Medicine III, University Hospital Carl Gustav Carus Dresden, Technische Universität Dresden, Dresden, Germany

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Michael Lauseker Institute for Medical Information Processing, Biometry, and Epidemiology (IBE), Ludwig Maximilian University of Munich, Munich, Germany

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Julian Maurer Department of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany

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Astrid Reul Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH), Zurich, Switzerland

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Gerald Spoettl Department of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany

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Barbara Klink Institute for Clinical Genetics, Faculty of Medicine Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany
National Center of Genetics, Laboratoire National de Santé, Dudelange, Luxembourg
German Cancer Consortium, Dresden, Germany

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Doreen William German Cancer Consortium, Dresden, Germany

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Thomas Knösel Institute of Pathology, Ludwig Maximilian University of Munich, Munich, Germany

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Juliane Friemel Department of Pathology and Molecular Pathology, University Hospital Zurich, Zurich, Switzerland

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Michel Bihl Department of Pathology and Molecular Pathology, University Hospital Zurich, Zurich, Switzerland

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Achim Weber Department of Pathology and Molecular Pathology, University Hospital Zurich, Zurich, Switzerland

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Maria Fankhauser Department of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany

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Laura Schober Department of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany

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Diana Vetter Department of Visceral and Transplantation Surgery, University Hospital, Zurich, Switzerland

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Martina Broglie Däppen Department of Otorhinolaryngology, University Hospital Zurich, Zurich, Switzerland

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

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

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

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

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Christian Lottspeich Department of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany

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Matthias Kroiss Department of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany
Division of Endocrinology and Diabetes, Department of Medicine I, University Hospital Würzburg, University of Würzburg, Würzburg, Germany

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Martin Fassnacht Division of Endocrinology and Diabetes, Department of Medicine I, University Hospital Würzburg, University of Würzburg, Würzburg, Germany

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Vera Ursula Julia Wenter Department of Nuclear Medicine, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany

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Roland Ladurner Department of General-, Visceral-, and Transplant-Surgery, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany

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Constanze Hantel Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH), Zurich, Switzerland
Department of Internal Medicine III, University Hospital Carl Gustav Carus Dresden, Technische Universität Dresden, Dresden, Germany

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Martin Reincke Department of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany

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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 Internal Medicine III, University Hospital Carl Gustav Carus Dresden, Technische Universität Dresden, Dresden, Germany

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Ashley B Grossman Green Templeton College, University of Oxford, Oxford, UK
NET Unit, ENETS Centre of Excellence, Royal Free Hospital, London, UK

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

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Felix Beuschlein Department of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany
Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH), Zurich, Switzerland

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Christoph J Auernhammer Department of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany

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Natalia S Pellegata Institute for Diabetes and Cancer (IDC), Helmholtz Center Munich, German Research Center for Environmental Health, Neuherberg, Germany

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Svenja Nölting Department of Internal Medicine IV, University Hospital, LMU Klinikum, Ludwig Maximilian University of Munich, Munich, Germany
Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ) and University of Zurich (UZH), Zurich, Switzerland

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-associated cluster 1 is subdivided into cluster 1A (Krebs cycle-related) and cluster 1B (hypoxia signaling related). Cluster 1-related mutations lead to hypoxia-inducible factor (HIF) 2α stabilization and accumulation which promote angiogenesis, tumor cell migration

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-21-0355
Volume/Issue:
Volume 29: Issue 6
Restricted access
P N Span Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine
Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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J U Rao Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine
Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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S B J Oude Ophuis Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine
Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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J W M Lenders Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine
Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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F C G J Sweep Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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P Wesseling Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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B Kusters Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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F H van Nederveen Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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R R de Krijger Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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A R M M Hermus Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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H J L M Timmers Department of Laboratory Medicine, Department of Radiation Oncology, Department of Endocrinology, Division of Vascular Medicine, Department of Pathology, Department of Pathology, Department of Internal Medicine III, Department of Internal Medicine

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). These ‘cluster 1’ tumors are distinct from ‘cluster 2’ tumors, which include RET - and NF1 -related and another subset of sporadic PGLs ( Dahia et al . 2005 ). This latter cluster is characterized by the deregulation of the RAS/RAF/MAP kinase

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-10-0184
Volume/Issue:
Volume 18: Issue 3
Free access
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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/PGLs into cluster 1 and 2 within the neuroendocrine tumor (NET) grading classification system, as seen in the classification of gastroenteropancreatic (GEP) and bronchopulmonary (BP) tumors ( Favier et al. 2015 ). NETs of the GEP and BP systems have a

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-21-0216
Volume/Issue:
Volume 28: Issue 11
Restricted access
Minghao Li Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, Fetscherstrasse, Dresden, Germany
Department of Urology, Xiangya Hospital, Central South University, Changsha, China

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

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Hermine Mohr Institute for Diabetes and Cancer, Helmholtz Centre Munich, Neuherberg, Germany

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Stephan Drukewitz Institute of Human Genetics, University of Leipzig Medical Center, Leipzig, Germany
Core Unit for Molecular Tumor Diagnostics (CMTD), National Center for Tumor Diseases (NCT), Partner Site Dresden, Dresden, Germany

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Isabel Poser Institute for Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany

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Daniela Stanke Institute for Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany

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Bruna Calsina Hereditary Endocrine Cancer Group, Spanish National Cancer Research Center and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain

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Angel M Martinez-Montes Hereditary Endocrine Cancer Group, Spanish National Cancer Research Center and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain

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Marcus Quinkler Endocrinology in Charlottenburg, Berlin, Germany

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

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Svenja Nölting Medizinische Klinik Und Poliklinik IV, Klinikum der Ludwig-Maximilians-Universität München, Munich, Germany
Department of Endocrinology, Diabetology and Clinical Nutrition, Universitätsspital Zürich (USZ) and University of Zurich (UZH), Zurich, Switzerland

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Felix Beuschlein Medizinische Klinik Und Poliklinik IV, Klinikum der Ludwig-Maximilians-Universität München, Munich, Germany
Department of Endocrinology, Diabetology and Clinical Nutrition, Universitätsspital Zürich (USZ) and University of Zurich (UZH), Zurich, Switzerland

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Hanna Remde Division of Endocrinology and Diabetes, Department of Internal Medicine I, University Hospital of Würzburg, Würzburg, Germany

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Giuseppe Opocher Department of Medicine, University of Padua, Padua, Italy

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

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

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Christina Pamporaki Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, Fetscherstrasse, Dresden, Germany

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Mercedes Robledo Hereditary Endocrine Cancer Group, Spanish National Cancer Research Center and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain

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Longfei Liu Department of Urology, Xiangya Hospital, Central South University, Changsha, China

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Jingjing Jiang Department of Endocrinology and Metabolism, Zhongshan Hospital, Fudan University, Shanghai, China

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

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Graeme Eisenhofer Department of Medicine III, University Hospital Carl Gustav Carus, Technische Universität Dresden, Fetscherstrasse, Dresden, Germany

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Stephanie M J Fliedner University Cancer Center Schleswig-Holstein, University Medical Center Schleswig-Holsten, Lübeck, Germany

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Nicole Bechmann Institute for Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany

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studies showed a strong genotype–phenotype correlation in PPGLs ( Eisenhofer et al. 2011 , Crona et al. 2019 ). Tumors with pathogenic variants (PVs) in genes that lead to activation of hypoxia signaling pathways (cluster 1), such as genes encoding

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-23-0230
Volume/Issue:
Volume 30: Issue 12
Restricted access
Katarina Edfeldt Department of Surgical Sciences, Uppsala University, SE-751 85 Uppsala, Sweden

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Peyman Björklund Department of Surgical Sciences, Uppsala University, SE-751 85 Uppsala, Sweden

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Göran Åkerström Department of Surgical Sciences, Uppsala University, SE-751 85 Uppsala, Sweden

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Gunnar Westin Department of Surgical Sciences, Uppsala University, SE-751 85 Uppsala, Sweden

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Per Hellman Department of Surgical Sciences, Uppsala University, SE-751 85 Uppsala, Sweden

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Peter Stålberg Department of Surgical Sciences, Uppsala University, SE-751 85 Uppsala, Sweden

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method ( Ma & Dai 2011 ). The expression data was reduced to three PCs that visualized three clusters ( Fig. 1 ). Cluster 1 contained 11 primary tumors; cluster 2 contained five LN metastases, and cluster 3 contained seven primary tumors, 12 LN metastases

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-10-0256
Volume/Issue:
Volume 18: Issue 4
Free access
Nicole Bechmann Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Fetscherstrasse, Dresden, Germany
Department of Medicine III, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Fetscherstrasse, Dresden, Germany
Department of Experimental Diabetology, German Institute of Human Nutrition Potsdam-Rehbruecke, Nuthetal, Germany
German Center for Diabetes Research (DZD), München-Neuherberg, Germany

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Mats Leif Moskopp Department of Neurosurgery, Vivantes Friedrichshain Hospital, Charité Academic Teaching Hospital, Landsberger Allee, Berlin, Germany
Institute of Physiology, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Fetscherstrasse, Dresden, Germany

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

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Bruna Calsina Hereditary Endocrine Cancer Group, Spanish National Cancer Research Centre (CNIO), Madrid, Spain
Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain

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

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

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Markus Friedemann Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Fetscherstrasse, Dresden, Germany

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Katharina Langton Department of Medicine III, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Fetscherstrasse, Dresden, Germany

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

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

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Svenja Nölting Department of Medicine IV, University Hospital, LMU Munich, Munich, Germany

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Felix Beuschlein Department of Medicine IV, University Hospital, LMU Munich, Munich, Germany
Department of Endocrinology, Diabetology and Clinical Nutrition, UniviersitätsSpital Zürich, Zurich, Switzerland

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Martin Fassnacht Division of Endocrinology and Diabetes, Department of Internal Medicine I, University Hospital of Würzburg, University of Würzburg, Würzburg, Germany

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

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

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Hans K Ghayee Department of Medicine, Division of Endocrinology, University of Florida College of Medicine and Malcom Randall VA Medical Center, Gainesville, Florida, USA

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

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Peter Dieterich Institute of Physiology, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Fetscherstrasse, Dresden, Germany

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

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Ben Wielockx Institute of Clinical Chemistry and Laboratory Medicine, University Hospital Carl Gustav Carus, Medical Faculty Carl Gustav Carus, Technische Universität Dresden, Fetscherstrasse, Dresden, Germany

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Mercedes Robledo Hereditary Endocrine Cancer Group, Spanish National Cancer Research Centre (CNIO), Madrid, Spain
Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain

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Nan Qin Division of Pediatric Neuro-Oncogenomics, German Cancer Research Center (DKFZ), Heidelberg, Germany
German Consortium for Translational Cancer Research (DKTK), partner site Essen/Düsseldorf, Düsseldorf, Germany
Department of Pediatric Oncology, Hematology, and Clinical Immunology, Medical Faculty, University Hospital Düsseldorf, Düsseldorf, Germany
Department of Neuropathology, Medical Faculty, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany

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

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( Fishbein et al. 2017 , Fliedner et al. 2018 , Crona et al. 2019 ). PPGLs with activation of pseudohypoxic pathways (cluster-1) comprise those with mutations in genes encoding the von Hippel–Lindau ( VHL ) tumor suppressor, succinate dehydrogenase

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-20-0205
Volume/Issue:
Volume 27: Issue 11
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Fredrika Svahn Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden

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Karolina Solhusløkk Höse Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden

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Adam Stenman Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden
Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
Department of Breast, Endocrine Tumors and Sarcoma, Karolinska University Hospital, Stockholm, Sweden

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Yaxuan Liu Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden
Department of Breast Surgery, Obstetrics and Gynecology Hospital of Fudan University, Shanghai, China

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Jan Calissendorff Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden

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Emma Tham Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
Department of Clinical Genetics, Karolinska University Hospital, Stockholm, Sweden

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Ákos Végvári Division of Chemistry I, Department of Medical Biochemistry and Biophysics, Karolinska Institutet, Stockholm, Sweden

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Roman A Zubarev Division of Chemistry I, Department of Medical Biochemistry and Biophysics, Karolinska Institutet, Stockholm, Sweden

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Na Wang Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden
Department of Medicine Huddinge, Karolinska Institutet, Huddinge, Sweden

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Reju Korah Yale Endocrine Neoplasia Laboratory, Department of Surgery, Yale School of Medicine, New Haven, Connecticut, USA

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Tobias Carling Yale Endocrine Neoplasia Laboratory, Department of Surgery, Yale School of Medicine, New Haven, Connecticut, USA
Carling Adrenal Center, Tampa, Florida, USA

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Jan Zedenius Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
Department of Breast, Endocrine Tumors and Sarcoma, Karolinska University Hospital, Stockholm, Sweden

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Robert Bränström Department of Molecular Medicine and Surgery, Karolinska Institutet, Stockholm, Sweden
Department of Breast, Endocrine Tumors and Sarcoma, Karolinska University Hospital, Stockholm, Sweden

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C Christofer Juhlin Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden
Department of Clinical Pathology and Cancer Diagnostics, Karolinska University Hospital Stockholm, Sweden

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Catharina Larsson Department of Oncology-Pathology, Karolinska Institutet, Stockholm, Sweden

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al. 2022 ). Based on gene expression profiling, three molecular subgroups of PPGL have been identified ( Fishbein et al. 2017 , Juhlin 2021 ). Cluster 1 tumors demonstrate a pseudohypoxia expression profile, exhibit a hypermethylation phenotype

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-23-0061
Volume/Issue:
Volume 31: Issue 9
Open access
Lucie Evenepoel Pole of Cardiovascular Research, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium
Human Molecular Genetics, de Duve Institute, Université catholique de Louvain, Brussels, Belgium

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Raphaël Helaers Human Molecular Genetics, de Duve Institute, Université catholique de Louvain, Brussels, Belgium

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Laurent Vroonen Department of Endocrinology, Centre Hospitalier Universitaire de Liège, University of Liège, Domaine Universitaire du Sart-Tilman, Liège, Belgium

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Selda Aydin Pathology Department, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium

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Marc Hamoir Otolaryngology Department, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium

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Dominique Maiter Endocrinology Department, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium

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Miikka Vikkula Human Molecular Genetics, de Duve Institute, Université catholique de Louvain, Brussels, Belgium

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Alexandre Persu Pole of Cardiovascular Research, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium
Cardiology Department, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium

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SDHB and 1 MAX ) ( Fig. 1 ). Using gene expression profiling, PPGLs can be classified in two clusters: Cluster 1 (hypoxia cluster) containing SDHx -, FH - and VHL -related tumors, and Cluster 2 (kinase signaling cluster) including MAX- , NF1

Article Type:
Letter
DOI:
https://doi.org/10.1530/ERC-17-0061
Volume/Issue:
Volume 24: Issue 8
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Marta Vaquero Department of Experimental Medicine, Universitat de Lleida/Institut de Recerca Biomèdica de Lleida, Lleida, Spain

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Anna Macià Department of Experimental Medicine, Universitat de Lleida/Institut de Recerca Biomèdica de Lleida, Lleida, Spain

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Carlos Anerillas Department of Experimental Medicine, Universitat de Lleida/Institut de Recerca Biomèdica de Lleida, Lleida, Spain

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Ana Velasco Department of Pathology, Universitat de Lleida/Institut de Recerca Biomèdica de Lleida, Lleida, Spain

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Xavier Matias-Guiu Department of Pathology, Universitat de Lleida/Institut de Recerca Biomèdica de Lleida, Lleida, Spain

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Joan Ribera Department of Experimental Medicine, Universitat de Lleida/Institut de Recerca Biomèdica de Lleida, Lleida, Spain

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Mario Encinas Department of Experimental Medicine, Universitat de Lleida/Institut de Recerca Biomèdica de Lleida, Lleida, Spain
Department of Pathology, Universitat de Lleida/Institut de Recerca Biomèdica de Lleida, Lleida, Spain

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clusters characterized by distinct expression signatures ( Gimenez-Roqueplo et al. 2012 ). Cluster 1 (comprising mutations of SDHx , FH , VHL , or HIF2A ( EPAS1 )) presents a pseudo-hypoxia signature characterized by a switch from oxidative

Article Type:
Letter
DOI:
https://doi.org/10.1530/ERC-15-0585
Volume/Issue:
Volume 23: Issue 4
Free access