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Esther Korpershoek
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Bart-Jeroen Petri
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Francien H van Nederveen
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Winand N M Dinjens
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Albert A Verhofstad
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Wouter W de Herder
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Sonja Schmid
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Aurel Perren
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Paul Komminoth
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Ronald R de Krijger
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. Gimenez-Roqueplo AP , Favier J, Rustin P, Rieubland C, Crespin M, Nau V, Khau Van Kien P, Corvol P, Plouin PF & Jeunemaitre X 2003 Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas. Cancer Research

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Kjell Oberg Department of Endocrine Oncology, University Hospital, Entrance 78, SE-751 85 Uppsala, Sweden

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implications ( Rindi et al . 2000 , Turner et al . 2006 , Vinik et al . 2009 ). Neuroendocrine cells can be confined to a specific gland, such as the adrenal medulla (phaeochromocytomas) or the thyroid (medullary thyroid carcinoma). These tumours will not

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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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.1186/1471-2350-10-34 ) 19368708 10.1186/1471-2350-10-34 Bayley JP Kunst HP Cascon A Sampietro ML Gaal J Korpershoek E Hinojar-Gutierrez A Timmers HJ Hoefsloot LH Hermsen MA , et al . 2010 SDHAF2 mutations in familial and sporadic paraganglioma and phaeochromocytoma . Lancet

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Arthur S Tischler Department of Pathology and Laboratory Medicine, Department of Pathology, Department of Pathology, Tufts Medical Center, Tufts University School of Medicine, 800 Washington Street, Box 802, Boston, Massachusetts 02111, USA

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Ronald R deKrijger Department of Pathology and Laboratory Medicine, Department of Pathology, Department of Pathology, Tufts Medical Center, Tufts University School of Medicine, 800 Washington Street, Box 802, Boston, Massachusetts 02111, USA
Department of Pathology and Laboratory Medicine, Department of Pathology, Department of Pathology, Tufts Medical Center, Tufts University School of Medicine, 800 Washington Street, Box 802, Boston, Massachusetts 02111, USA

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) , and the Grading system for Adrenal Phaeochromocytoma and Paraganglioma (GAPP), proposed by Kimura et al . (2014) ( Fig. 4 ). While PASS applies only to pheochromocytomas, GAPP applies to both pheochromocytomas and paragangliomas. Morphological

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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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-sensing defect . Human Genetics 113 228 – 237 . ( doi:10.1007/s00439-003-0969-6 ). Astuti D Douglas F Lennard TW Aligianis IA Woodward ER Evans DG Eng C Latif F Maher ER 2001a Germline SDHD mutation in familial phaeochromocytoma

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Graeme Eisenhofer
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Stefan R Bornstein
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Frederieke M Brouwers
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Nai-Kong V Cheung
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Patricia L Dahia
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Ronald R de Krijger
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Thomas J Giordano
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Lloyd A Greene
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David S Goldstein
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Hendrik Lehnert
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William M Manger
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John M Maris
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Hartmut P H Neumann
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Karel Pacak
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Barry L Shulkin
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David I Smith
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Arthur S Tischler
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William F Young Jr
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Hereditary phaeochromocytoma and paragangliomas: a study of five susceptibility genes. Journal of Medical Genetics 40 e75 . Benn DE , Dwight T, Richardson AL, Delbridge L, Bambach CP, Stowasser M, Gordon RD, Marsh

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Dorota Dworakowska Barts and the London School of Medicine, Department of Endocrinology and Internal Medicine, Centre for Endocrinology, London EC1M 6BQ, UK
Barts and the London School of Medicine, Department of Endocrinology and Internal Medicine, Centre for Endocrinology, London EC1M 6BQ, UK

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Ashley B Grossman Barts and the London School of Medicine, Department of Endocrinology and Internal Medicine, Centre for Endocrinology, London EC1M 6BQ, UK

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-entero-pancreatic and bronchial NETs and phaeochromocytomas and paragangliomas. They may occur sporadically or in a familial context of autosomal dominant inherited syndromes such as multiple endocrine neoplasia (MEN1 and MEN2), VHL, Carney complex (principally

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Katherine I Wolf Department of Medicine, Division of Metabolism, Endocrinology and Diabetes, University of Michigan, Ann Arbor, Michigan, USA

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Linda Rose-Krasnor Pheo Para Alliance and Psychology Department, Brock University, St. Catharines, Ontario, Canada

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Stephanie Alband Pheo Para Alliance, Alexandria, Virginia, USA

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Jacques W M Lenders Department of Internal Medicine, Radboud University Medical Centre, Nijmegen, The Netherlands

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Lauren Fishbein Department of Medicine, Division of Endocrinology, Metabolism and Diabetes, and Department of Biomedical Informatics, University of Colorado School of Medicine, Aurora, Colorado, USA

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their journey in hopes of advancing awareness and research. References Andersen GS Toftdahl DB Lund JO Strandgaard S & Nielsen PE 1988 The incidence rate of phaeochromocytoma and Conn’s syndrome in Denmark, 1977–1981 . Journal of

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Gergely Huszty Department of Surgery, Transplantation and Gastroenterology, Faculty of Medicine, Semmelweis University, Budapest, Hungary

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Peter Igaz Department of Endocrinology, Faculty of Medicine, Semmelweis University, Budapest, Hungary
Department of Internal Medicine and Oncology, Faculty of Medicine, Semmelweis University, Budapest, Hungary

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.1016/j.ijsu.2022.106853 ) Lenders JWM Kerstens MN Amar L Prejbisz A Robledo M Taieb D Pacak K Crona J Zelinka T Mannelli M , et al. 2020 Genetics, diagnosis, management and future directions of research of phaeochromocytoma and

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Marta Araujo-Castro Endocrinology & Nutrition Department, Hospital Universitario Ramón y Cajal, Madrid, Spain
Instituto de Investigación Biomédica Ramón y Cajal (IRYCIS), Madrid, Spain
University of Alcalá, Madrid, Spain

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César Mínguez Ojeda Urology Department, Hospital Universitario Ramón y Cajal, Madrid, Spain

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Rogelio García Centeno Endocrinology & Nutrition Department, Hospital Universitario Gregorio Marañón, Madrid, Spain

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María-Carmen López-García Endocrinology & Nutrition Department, Hospital Universitario de Albacete, Albacete, Spain

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Cristina Lamas Endocrinology & Nutrition Department, Hospital Universitario de Albacete, Albacete, Spain

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Felicia Alexandra Hanzu Endocrinology & Nutrition Department, Hospital Clinic, Barcelona, Spain

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Mireia Mora Endocrinology & Nutrition Department, Hospital Clinic, Barcelona, Spain

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María del Castillo Tous Endocrinology & Nutrition Department, Hospital Universitario Virgen de la Macarena, Sevilla, Spain

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Pablo Rodríguez de Vera Gómez Endocrinology & Nutrition Department, Hospital Universitario Virgen de la Macarena, Sevilla, Spain

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Paola Parra Ramírez Endocrinology & Nutrition Department, Hospital Universitario La Paz Madrid, Madrid, Spain

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Cristina Alvarez-Escola Endocrinology & Nutrition Department, Hospital Universitario La Paz Madrid, Madrid, Spain

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Concepción Blanco Carrera Endocrinology & Nutrition Department, Hospital Universitario Príncipe de Asturias, Madrid, Spain

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Rebeca Barahona San Millán Endocrinology & Nutrition Department, Institut Català de la Salut Girona, Girona, Spain

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Mónica Recasens Endocrinology & Nutrition Department, Institut Català de la Salut Girona, Girona, Spain

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Nuria Valdés Endocrinology & Nutrition Department, Hospital Universitario de Cabueñes, Asturias, Spain

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Paola Gracia Gimeno Endocrinology & Nutrition Department, Hospital Royo Villanova, Zaragoza, Spain

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Paz de Miguel Novoa Endocrinology & Nutrition Department, Hospital Clínico San Carlos, Madrid, Spain

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Almudena Vicente Endocrinology & Nutrition Department, Hospital Universitario de Toledo, Toledo, Spain

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Laura Manjón Endocrinology & Nutrition Department, Hospital Universitario Central de Asturias, Oviedo, Spain
Instituto de Investigación Sanitaria del Principado de Asturias (ISPA)

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Iñigo García Sanz General & Digestive Surgery Department, Hospital Universitario de La Princesa, Madrid, Spain

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Theodora Michalopoulou Department of Endocrinology and Nutrition, Joan XXIII University Hospital, Tarragona, Spain

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María Calatayud Endocrinology & Nutrition Department, Hospital Universitario Doce de Octubre, Madrid, Spain

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had diabetes mellitus before surgery, with complete resolution in 79% ( n  = 33) after extirpation of the phaeochromocytoma. Similarly, Liu et al . (2019) described a remission rate of diabetes of 70%, with high BMI as a risk factor for persistent

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