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Liang Zhang Department of Medical Sciences, Uppsala University, Uppsala, Sweden

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Tobias Åkerström Department of Surgical Sciences, Uppsala University, Uppsala, Sweden

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Kazhan Mollazadegan Department of Medical Sciences, Uppsala University, Uppsala, Sweden

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Felix Beuschlein Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, Universitätsspital Zürich (USZ) and Univeristät Zürich (UZH), Zurich, Switzerland
Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Munich, Germany

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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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Britt Skogseid Department of Medical Sciences, Uppsala University, Uppsala, Sweden

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Joakim Crona Department of Medical Sciences, Uppsala University, Uppsala, Sweden

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Dear Editor, We greatly appreciate the comments by Gergely Huszty and Peter Igaz, published in this issue of Endocrine-Related Cancer article ID e230220 , on our review ‘Risk of complications after core needle biopsy in pheochromocytoma/paraganglioma

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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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Dear Editor, Pheochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors arising from the adrenal medulla and extra-adrenal paraganglia, respectively. Forty percent are explained by germline mutations in known susceptibility

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Eamonn R Maher Department of Medical Genetics, Department of Clinical Genetics, University of Cambridge, Cambridge, UK and
Department of Medical Genetics, Department of Clinical Genetics, University of Cambridge, Cambridge, UK and

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oxygen-dependent prolyl hydroxylases are inactive, pVHL-dependent degradation of HIFα subunits is compromised and HIF1 and HIF2 are stabilised and activate downstream transcriptional pathways. A notable feature of pheochromocytoma and paraganglioma is the

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Adrian F Daly Department of Endocrinology Centre Hospitalier Universitaire de Liège, University of Liège, Domaine Universitaire du Sart‐Tilman, 4000 Liège, Belgium

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Albert Beckers Department of Endocrinology Centre Hospitalier Universitaire de Liège, University of Liège, Domaine Universitaire du Sart‐Tilman, 4000 Liège, Belgium

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stromal tumor (GIST; at the time gastric ‘leiomyosarcoma’), paraganglioma, and pulmonary chondroma. While it is traditional to use the term GIST in describing the gastric lesions in Carney triad, they are probably better described as ‘gastric stromal

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Hartmut P Neumann Section for Preventive Medicine, University Medical Center, Albert-Ludwigs-University, Freiburg, Germany

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William F Young Jr Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, New York, USA

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Tobias Krauss Department of Radiology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany

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Jean-Pierre Bayley Department of Human Genetics, Leiden University Medical Center, Leiden, The Netherlands

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Francesca Schiavi Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology, IRCCS, Padova, Italy

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Giuseppe Opocher Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology, IRCCS, Padova, Italy

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Carsten C Boedeker Department of Otorhinolaryngology, HELIOS Hanseklinikum Stralsund, Stralsund, Germany

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Amit Tirosh Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel

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Frederic Castinetti Department of Endocrinology, Aix-Marseille Université, Institut National de la Santé et de la Recherche Médicale (INSERM), U1251, Marseille Medical Genetics (MMG), Marseille, France
Assistance Publique – Hôpitaux de Marseille (AP-HM), Hôpital de la Conception, Centre de Référence des Maladies Rares Hypophysaires HYPO, Marseille, France

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Juri Ruf Department of Nuclear Medicine, Faculty of Medicine, Albert-Ludwigs-University, Freiburg, Germany

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Dmitry Beltsevich Department of Surgery, Endocrinology Research Center, Moscow, Russia

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Martin Walz Department of Surgery and Center of Minimally-Invasive Surgery, Kliniken Essen-Mitte, Essen, Germany

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Harald-Thomas Groeben Department of Anesthesiology, Kliniken Essen-Mitte, Essen, Germany

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Ernst von Dobschuetz Section of Endocrine Surgery, Clinic of General, Visceral and Thoracic Surgery, Krankenhaus Reinbek, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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

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Nelson Wohllk Endocrine Section, Universidad de Chile, Hospital del Salvador, Santiago de Chile, Chile

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Marija Pfeifer Department of Endocrinology, University Medical Center Ljubljana, Ljubljana, Slovenia

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Delmar M Lourenço Jr Endocrine Genetics Unit, Endocrinology Division, Hospital das Clínicas, University of São Paulo School of Medicine (FMUSP), Endocrine Oncology Division, Institute of Cancer of the State of São Paulo, FMUSP, São Paulo, Brazil

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Mariola Peczkowska Department of Hypertension, Institute of Cardiology, Warsaw, Poland

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Attila Patocs HSA-SE ‘Lendület’ Hereditary Endocrine Tumor Research Group, Hungarian Academy of Sciences and Semmelweis University, Budapest, Hungary

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Joanne Ngeow Lee Kong Chian School of Medicine, Nanyang Technological University Singapore and Cancer Genetics Service, National Cancer Centre Singapore, Singapore, Singapore

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Özer Makay Division of Endocrine Surgery, Department of General Surgery, Ege University, Izmir, Turkey

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Nalini S Shah Department of Endocrinology, Seth G S Medical College, K.E.M. Hospital, Parel, Mumbai, India

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

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Helena Leijon Department of Pathology, University of Helsinki, and HUSLAB, Helsinki University Hospital, Helsinki, Finland

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Gianmaria Pennelli Department of Medicine (DIMED), Surgical Pathology Unit, University of Padua, Padua, Italy

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Karina Villar Gómez de las Heras Central Services, Servicio de Salud de Castilla-La Mancha (SESCAM), Toledo, Spain

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Thera P Links Department of Endocrinology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands

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Birke Bausch Department of Medicine II, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany

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Charis Eng Genomic Medicine Institute, Lerner Research Institute and Taussig Cancer Institute, Cleveland Clinic, Cleveland, Ohio, USA

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cells or tumor, the middle syllables chromo refer to the former usage of a special chromate-containing stain and pheo refers to the classic brown appearance after exposure to chromate staining ( Bausch et al . 2017 b ). In contrast, paraganglioma

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Arthur Varoquaux Department of Radiology, Conception Hospital, Aix-Marseille University, Marseille, France

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Electron Kebebew Endocrine Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA

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Fréderic Sebag Department of Endocrine Surgery, Conception Hospital, Aix-Marseille University, Marseille, France

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Katherine Wolf Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health, Bethesda, Maryland, USA

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Jean-François Henry Department of Endocrine Surgery, Conception Hospital, Aix-Marseille University, Marseille, France

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

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David Taïeb Department of Nuclear Medicine, La Timone University Hospital, CERIMED, Aix-Marseille University, Marseille, France

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third epibranchial placode (nodose placode) ( Baker 2005 ). Vagal paragangliomas Tumor origin and molecular genetics Vagus nerve paraganglia belong to the family of parasympathetic paraganglia. Members act as chemoreceptors and are involved

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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, Tufts University School of Medicine, Boston, Massachusetts, USA

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James D Baleja Department of Developmental, Molecular and Chemical Biology, Tufts University School of Medicine, Boston, Massachusetts, USA

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

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Xue Zhang Department of Developmental, Molecular and Chemical Biology, Tufts University School of Medicine, Boston, Massachusetts, USA

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

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Introduction SDH-deficient gastrointestinal stromal tumors (GISTs) are rare but sometimes lethal tumors that can occur in patients with paraganglioma (PGL) syndromes. These tumors do not harbor the KIT or PDGFRA mutations typical of

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Michael Solarski Division of Neurosurgery, Department of Surgery, St. Michael’s Hospital, Toronto, Ontario, Canada

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Fabio Rotondo Division of Pathology, Department of Laboratory Medicine, St. Michael’s Hospital, Toronto, Ontario, Canada

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William D Foulkes Department of Human Genetics, Medicine and Oncology, McGill University, Montreal, Quebec, Canada
Lady Davis Institute, Jewish General Hospital and Research Institute, McGill University Health Centre, Montreal, Quebec, Canada

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John R Priest Department of Medicine, Minneapolis, Minnesota, USA

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Luis V Syro Department of Neurosurgery, Hospital Pablo Tobon Uribe and Clinica Medellin, Medellin, Colombia

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Henriett Butz Molecular Medicine Research Group, Hungarian Academy of Sciences, Semmelweis University, Budapest, Hungary

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Michael D Cusimano Division of Neurosurgery, Department of Surgery, St. Michael’s Hospital, Toronto, Ontario, Canada

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Kalman Kovacs Division of Pathology, Department of Laboratory Medicine, St. Michael’s Hospital, Toronto, Ontario, Canada

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treatments ( Terzolo et al . 2014 ). Whether or not DICER1 mutations are present in adrenocortical cancer remains to be seen, but they could present a much-needed therapeutic target for this disease. DICER1 mutations in paragangliomas Similar to

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M Montani
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A M Schmitt
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S Schmid
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T Locher
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P Saremaslani
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P U Heitz
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P Komminoth
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A Perren
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. 2001 ) have recently been described in familial paragangliomas and pheochromocytomas. Several reasons make these genes candidate tumor suppressors for MTC: (1) allelic loss of the short and long arm of chromosome 1, the localization of SDHB and SDHC

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Robin Schürfeld Division of Endocrinology and Diabetes, Department of Internal Medicine, University of Leipzig, Leipzig, Germany

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Christina Pamporaki TU Dresden, Medical Clinic III, University Hospital Carl Gustav Carus, Dresden, Germany

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Mirko Peitzsch TU Dresden, Institute of Clinical Chemistry and Laboratory Medicine, Dresden, Germany

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Nada Rayes Center of Surgery, Division of Endocrine Surgery, Department for Visceral, Transplant, Thoracic, and Vascular Surgery, University of Leipzig, Leipzig, Germany

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Osama Sabri Department of Nuclear Medicine, University of Leipzig, Leipzig, Germany

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Silvio Rohm Center of Surgery, Department for Visceral, Transplant, Thoracic, and Vascular Surgery, University of Leipzig, Leipzig, Germany
Center of Surgery, Department for Vascular Surgery, Diakonissen Hospital of Leipzig, Leipzig, Germany

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Ronald Biemann Institute of Clinical Chemistry and Laboratory Medicine, University of Leipzig, Leipzig, Germany

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Benjamin Sandner Division of Endocrinology and Diabetes, Department of Internal Medicine, University of Leipzig, Leipzig, Germany

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Anke Tönjes Division of Endocrinology and Diabetes, Department of Internal Medicine, University of Leipzig, Leipzig, Germany

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Graeme Eisenhofer TU Dresden, Medical Clinic III, University Hospital Carl Gustav Carus, Dresden, Germany

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Introduction Pheochromocytomas and paragangliomas (PPGL) are neuroendocrine tumors derived from chromaffin cells of the adrenal medulla or extraparaganglionic tissue ( Tischler 2008 ). Measurements of plasma-free normetanephrine and

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