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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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Introduction Paragangliomas (PGLs) are neuroendocrine tumors that originate from neural crest-derived cells of the sympathetic and parasympathetic ganglia. PGLs that arise from chromaffin cells of the adrenal medulla are named

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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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V H M Tsang Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research
Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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T Dwight Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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D E Benn Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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G Y Meyer-Rochow Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research
Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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A J Gill Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research
Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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M Sywak Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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S Sidhu Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research
Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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D Veivers Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research
Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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C M Sue Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research
Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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B G Robinson Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research
Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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R J Clifton-Bligh Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research
Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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N R Parker Cancer Genetics Laboratory, Department of Endocrinology, Department of Surgery, Department of Anatomical Pathology, Cancer Diagnosis and Oncology Group, Department of Endocrine and Oncology Surgery, Neurogenetics Research Laboratory, Department of ENT Surgery, Department of Neurology, Bill Walsh Translational Cancer Research Laboratory, Kolling Institute of Medical Research

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Introduction Pheochromocytomas (PCs) are catecholamine-secreting tumours of chromaffin cells of the adrenal medulla and are closely related to paragangliomas (PGLs) which arise from sympathetic and parasympathetic chains ( Favier et al . 2005

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Roland Därr 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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Joan Nambuba 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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Jaydira Del Rivero 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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Ingo Janssen 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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Maria Merino Laboratory of Pathology, National Institutes of Health, Bethesda, Maryland, USA

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Milena Todorovic Institute of Hematology, Clinical Center of Serbia and Medical Faculty University of Belgrade, Belgrade, Serbia

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Bela Balint Institute of Transfusiology and Hemobiology of Military Medical Academy and Institute for Medical Research, University of Belgrade, Belgrade, Serbia

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Ivana Jochmanova Section on Medical Neuroendocrinology, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA
1st Department of Internal Medicine, Faculty of Medicine, Pavol Jozef Safarik University in Kosice, Kosice, Slovakia

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Josef T Prchal Division of Hematology, University of Utah, Salt Lake City, Utah, USA

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Ronald M Lechan Tupper Research Institute and Department of Medicine, Division of Endocrinology, Diabetes and Metabolism, Tufts Medical Center, Boston, Massachusetts, USA

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

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Vera Popovic Institute of Endocrinology, Clinical Center of Serbia, Medical Faculty, University Belgrade, Belgrade, Serbia

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Dragana Miljic Institute of Endocrinology, Clinical Center of Serbia, Medical Faculty, University Belgrade, Belgrade, Serbia

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Karen T Adams 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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F Ryan Prall Department of Ophthalmology, Eugene and Marilyn Glick Eye Institute, Indiana University School of Medicine, Indianapolis, Indiana, USA

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Alexander Ling Department of Radiology and Imaging Sciences, Clinical Center, National Institutes of Health, Bethesda, Maryland, USA

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Meredith R Golomb Division of Child Neurology, Department of Neurology, Indiana University School of Medicine, Indianapolis, Indiana, USA

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Michael Ferguson Riley Hospital for Children at Indiana University Health, Indianapolis, Indiana, USA

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Naris Nilubol Endocrine Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA

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Clara C Chen Division of Nuclear Medicine, Department of Radiology and Imaging Sciences, Clinical Center, National Institutes of Health, Bethesda, Maryland, USA

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Emily Chew Division of Epidemiology and Clinical Applications, National Eye Institute, National Institutes of Health, Bethesda, Maryland, USA

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David Taïeb Department of Nuclear Medicine, La Timone University Hospital & CERIMED & Inserm UMR1068 Marseille Cancerology Research Center, Institut Paoli-Calmettes, Aix-Marseille University, Marseille, France

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Constantine A Stratakis Division of Intramural Research, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA

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Tito Fojo Medical Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA

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Chunzhang Yang Neuro-Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA

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

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Zhengping Zhuang Neuro-Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA

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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 Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are rare catecholamine-producing neuroendocrine tumors (NETs) arising in or outside the adrenal medulla, respectively ( Lenders et al . 2005 ). By definition, a PHEO is an intra

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

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Peter Devilee Department of Human Genetics, Leiden University Medical Center, Leiden, the Netherlands
Department of Pathology, Leiden University Medical Center, Leiden, the Netherlands

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Introduction Paraganglioma–pheochromocytoma Paragangliomas and pheochromocytomas are neuroendocrine tumors that arise mainly in the adrenal medulla or paraganglia of the head and neck, but may also develop in abdominal or thoracic

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Adam I Kaplan Kolling Institute of Medical Research, The University of Sydney, Sydney, Australia
Department of Endocrinology, Royal North Shore Hospital, Sydney, Australia

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Trisha Dwight Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, Sydney, Australia
Faculty of Medicine and Health, The University of Sydney, Sydney, Australia

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Catherine Luxford Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, Sydney, Australia
Faculty of Medicine and Health, The University of Sydney, Sydney, Australia

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Diana E Benn Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, Sydney, Australia
Faculty of Medicine and Health, The University of Sydney, Sydney, Australia

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Roderick J Clifton-Bligh Department of Endocrinology, Royal North Shore Hospital, Sydney, Australia
Cancer Genetics Laboratory, Kolling Institute, Royal North Shore Hospital, Sydney, Australia
Faculty of Medicine and Health, The University of Sydney, Sydney, Australia

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Introduction Phaeochromocytomas and paragangliomas (collectively termed PPGL) are rare neuroendocrine tumours originating from chromaffin cells of the adrenal medulla, and neuroendocrine paraganglia, respectively. Paragangliomas (PGLs) are

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N Tufton Centre for Endocrinology, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Charterhouse Square, London, UK
Department of Endocrinology, St Bartholomew’s Hospital, Barts Health NHS Trust. West Smithfield, London, UK

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R J Hearnden Centre for Endocrinology, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Charterhouse Square, London, UK

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D M Berney Department of Pathology, Royal London Hospital, Whitechapel, London, UK

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W M Drake Centre for Endocrinology, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Charterhouse Square, London, UK
Department of Endocrinology, St Bartholomew’s Hospital, Barts Health NHS Trust. West Smithfield, London, UK

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L Parvanta Department of Endocrine Surgery, St Bartholomew’s Hospital, Barts Health NHS Trust, West Smithfield, London, UK

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J P Chapple Centre for Endocrinology, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Charterhouse Square, London, UK

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S A Akker Centre for Endocrinology, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, Charterhouse Square, London, UK
Department of Endocrinology, St Bartholomew’s Hospital, Barts Health NHS Trust. West Smithfield, London, UK

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Introduction Phaeochromocytoma and paragangliomas (PPGLs) are rare neuroendocrine tumours of the autonomic nervous system that can arise anywhere from the skull base to the pelvic floor and may secrete catecholamines. All PPGLs should be

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