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Anne-Paule Gimenez-Roqueplo Université Paris Cité, PARCC, INSERM, Paris, France
Département de Médecine Génomique des Tumeurs et des Cancers, Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France

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Mercedes Robledo Hereditary Endocrine Cancer Group, Human Cancer Genetics Programme, Spanish National Cancer Research Centre (CNIO), Madrid, Spain
Biomedical Research Networking Centre on Rare Diseases (CIBERER), Institute of Health Carlos III, Madrid, Spain

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Patricia L M Dahia Division of Hematology and Medical Oncology, Department Medicine, University of Texas Health Science Center at San Antonio (UTHSCSA), San Antonio, Texas, USA
Mays Cancer Center at UTHSCSA, San Antonio, Texas, USA

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delays caused by the COVID-19 pandemic. In the intervening years, two international meetings related to pheochromocytomas (PCC) and paragangliomas (PGL) took place, Multiple Endocrine Neoplasia Workshop 2019 (in Houston) and 2021 (virtual). In 2019, the

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Samuel M O'Toole Department of Endocrinology, Hereditary Endocrine Cancer Group, Section on Endocrinology and Genetics, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK

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Judit Dénes Department of Endocrinology, Hereditary Endocrine Cancer Group, Section on Endocrinology and Genetics, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK

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Mercedes Robledo Department of Endocrinology, Hereditary Endocrine Cancer Group, Section on Endocrinology and Genetics, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK

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Constantine A Stratakis Department of Endocrinology, Hereditary Endocrine Cancer Group, Section on Endocrinology and Genetics, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK

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Márta Korbonits Department of Endocrinology, Hereditary Endocrine Cancer Group, Section on Endocrinology and Genetics, Barts and the London School of Medicine, Queen Mary University of London, London EC1M 6BQ, UK

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Introduction Pituitary adenomas (PA) and phaeochromocytomas/paragangliomas (phaeo/PGL) are relatively rare tumours. The prevalence of symptomatic PA in the general population is around 1 in 1000 ( Daly et al . 2006 , Fernandez et al . 2010 ). The

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Diana E Benn Cancer Genetics, Kolling Institute, Royal North Shore Hospital, University of Sydney, St Leonards, New South Wales 2065, Australia

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Bruce G Robinson Cancer Genetics, Kolling Institute, Royal North Shore Hospital, University of Sydney, St Leonards, New South Wales 2065, Australia

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Roderick J Clifton-Bligh Cancer Genetics, Kolling Institute, Royal North Shore Hospital, University of Sydney, St Leonards, New South Wales 2065, Australia

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Introduction Phaeochromocytomas (PCs) are tumours in the adrenal medulla, and paragangliomas (PGLs) arise in extra-adrenal sympathetic chromaffin tissue or head and neck parasympathetic tissues. Familial occurrence of PGLs was first reported in 1933

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Camilo Jimenez University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Bennett B Chin University of Colorado Anschutz Medical Campus, Aurora, Colorado, USA

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Richard B Noto Warren Alpert Medical School of Brown University, Providence, Rhode Island, USA

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Joseph S Dillon University of Iowa, Iowa City, Iowa, USA

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Lilja Solnes Johns Hopkins Medicine, Baltimore, Maryland, USA

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Nancy Stambler Progenics Pharmaceuticals, Inc., a Lantheus Company, North Billerica, Massachusetts, USA

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Vincent A DiPippo Progenics Pharmaceuticals, Inc., a Lantheus Company, North Billerica, Massachusetts, USA

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Daniel A Pryma Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, USA

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Introduction Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from chromaffin cells of the adrenal medulla and paraganglia, respectively ( Jimenez 2018 ). Each year, approximately 500–1600 new cases of PPGLs

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Arthur Varoquaux Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France

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Yann le Fur Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France

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Alessio Imperiale Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France
Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France

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Antony Reyre Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France

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Marion Montava Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France

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Nicolas Fakhry Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France

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Izzie-Jacques Namer Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France
Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France

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Guy Moulin Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France

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Karel Pacak Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France

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Maxime Guye Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France
Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France

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David Taïeb Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France
Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France
Department of Medical Imaging, CNRS, Department of Biophysics and Nuclear Medicine, ICube, Department of Otorhinolaryngology–Head and Neck Surgery, Department of Otorhinolaryngology–Head and Neck Surgery, Program in Reproductive and Adult Endocrinology, Department of Nuclear Medicine, Biophysics and Nuclear Medicine, INSERM, La Timone University Hospital, Aix‐Marseille University, Marseille, France

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Introduction Paragangliomas (PGLs) are slow-growing hypervascular tumors arising from neural crest cell derivatives throughout the body. PGLs are closely aligned with the distribution of the autonomic nervous system and preferentially arise in the

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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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Introduction Pheochromocytomas (PCCs) and paragangliomas (PGLs, together denoted as PPGLs) are rare endocrine tumors originating from chromaffin cells in the adrenal medulla (PCC) and extra-adrenal paraganglia (PGL). Sympathetic PPGLs secrete

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Joakim Crona Department of Medical Sciences, Uppsala University, Uppsala, Sweden
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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Angela Lamarca Department of Medical Oncology, The Christie NHS Foundation Trust (ENETS Centre of Excellence), Manchester, UK

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Suman Ghosal 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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Staffan Welin Department of Medical Sciences, Uppsala University, Uppsala, Sweden

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

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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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Introduction The Cancer Genome Atlas (TCGA) proposed that neuroendocrine tumors of adrenal paraganglia, pheochromocytomas (PCCs) and extra-adrenal paraganglia paragangliomas (PGLs, together denoted PPGL) can be divided into three main

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Aguirre A de Cubas Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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L Javier Leandro-García Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Francesca Schiavi Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Veronika Mancikova Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Iñaki Comino-Méndez Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain
Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Lucía Inglada-Pérez Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain
Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Manuel Perez-Martinez Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Nuria Ibarz Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Pilar Ximénez-Embún Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Elena López-Jiménez Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Agnieszka Maliszewska Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Rocío Letón Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Álvaro Gómez Graña Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Carmen Bernal Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Cristina Álvarez-Escolá Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Cristina Rodríguez-Antona Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain
Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Giuseppe Opocher Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain
Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Javier Muñoz Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Diego Megias Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Alberto Cascón Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain
Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Mercedes Robledo Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain
Hereditary Endocrine Cancer Group, Familial Cancer Clinic, Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Confocal Microscopy Unit, Proteomics Unit, Endocrinology and Nutrition Service, Endocrinology and Nutrition Service, Department of Medical and Surgical Sciences, Human Cancer Genetics Programme, Spanish National Cancer Research Centre, Centro Nacional de Investigaciones Oncológicas, Melchor Fernández Almagro 3, 28029 Madrid, Spain

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Introduction Pheochromocytomas (PCCs) and paragangliomas (PGLs) are rare neuroendocrine tumors of neural crest origin. Up to an estimated 60% of PCCs/PGLs are associated with germline or somatic mutations in one many susceptibility genes ( RET

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Tobias Else Metabolism, Endocrinology and Diabetes, Department of Internal Medicine, University of Michigan, 2560E MSRB2, 1150 West Medical Center Drive, Ann Arbor, Michigan 48109‐5674, USA

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Introduction The last 15 years have brought significant advances in our understanding of the genetic basis of hereditary syndromes with a predisposition to pheochromocytoma (PC) and paraganglioma (PGL) (PCPGL) development. In the 1990s the genetic

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Noriko Kimura
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Ryoichi Takayanagi Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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Nae Takizawa Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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Eiji Itagaki Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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Takayuki Katabami Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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Narihiko Kakoi Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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Hiromi Rakugi Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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Yukihiro Ikeda Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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Akiyo Tanabe Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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Takeshi Nigawara Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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Sadayoshi Ito Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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Itaru Kimura Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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Mitsuhide Naruse Pathology Division, Department of Medicine and Bioregulatory Science, Department of Urology and Andrology, Third Department of Internal Medicine (Diabetes Endocrine and Metabolism), Department of Medicine, Department of Urology, Department of Geriatric Medicine and Nephrology, Division of Nephrology, Department of Endocrinology and Metabolism, Department of Endocrinology and Metabolism, Division of Nephrology, Council Member Examination Committee of Social Insurance, Department of Endocrinology, Department of Clinical Research, National Hospital Organization, Hakodate Hospital, Postal cord 041-8512, 18-16 Kawahara, Hakodate, Hokkaido, Japan

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The Phaeochromocytoma Study Group in Japan
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Introduction Phaeochromocytomas (PHEO) of the adrenal gland and sympathetic paragangliomas (PGL) are catecholamine-producing tumours. Although 10–30% of these tumours metastasise, histopathological criteria that discriminate malignant from benign

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