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V Häyry Institute of Biomedicine, Departments of Pathology, Otorhinolaryngology, Developmental Biology, University of Helsinki, Biomedicum, Haartmaninkatu 8, PL 63, Helsinki 00014 HY, Finland
Institute of Biomedicine, Departments of Pathology, Otorhinolaryngology, Developmental Biology, University of Helsinki, Biomedicum, Haartmaninkatu 8, PL 63, Helsinki 00014 HY, Finland

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K Salmenkivi Institute of Biomedicine, Departments of Pathology, Otorhinolaryngology, Developmental Biology, University of Helsinki, Biomedicum, Haartmaninkatu 8, PL 63, Helsinki 00014 HY, Finland

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J Arola Institute of Biomedicine, Departments of Pathology, Otorhinolaryngology, Developmental Biology, University of Helsinki, Biomedicum, Haartmaninkatu 8, PL 63, Helsinki 00014 HY, Finland

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P Heikkilä Institute of Biomedicine, Departments of Pathology, Otorhinolaryngology, Developmental Biology, University of Helsinki, Biomedicum, Haartmaninkatu 8, PL 63, Helsinki 00014 HY, Finland

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C Haglund Institute of Biomedicine, Departments of Pathology, Otorhinolaryngology, Developmental Biology, University of Helsinki, Biomedicum, Haartmaninkatu 8, PL 63, Helsinki 00014 HY, Finland

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H Sariola Institute of Biomedicine, Departments of Pathology, Otorhinolaryngology, Developmental Biology, University of Helsinki, Biomedicum, Haartmaninkatu 8, PL 63, Helsinki 00014 HY, Finland
Institute of Biomedicine, Departments of Pathology, Otorhinolaryngology, Developmental Biology, University of Helsinki, Biomedicum, Haartmaninkatu 8, PL 63, Helsinki 00014 HY, Finland

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Introduction Phaeochromocytoma is a rare tumour of the adrenal medulla. We lack exact incidence figures in Finland, but in Sweden, the incidence is 2.1 per million ( Stenstrom & Svardsudd 1986 ). It usually arises from adrenergic cells in the

Article Type:
Research Article
DOI:
https://doi.org/10.1677/ERC-09-0049
Volume/Issue:
Volume 16: Issue 4
Free access
Graeme Eisenhofer Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany
Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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Karel Pacak Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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Thanh-Truc Huynh Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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Nan Qin Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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Gennady Bratslavsky Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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W Marston Linehan Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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Massimo Mannelli Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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Peter Friberg Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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Stefan K Grebe Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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Henri J Timmers Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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Stefan R Bornstein Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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Jacques W M Lenders Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany
Institute of Clinical Chemistry and Laboratory Medicine, Department of Medicine, Reproductive and Adult Endocrinology Program, Urologic Oncology Branch, Department of Clinical Pathophysiology, Department of Clinical Physiology, Department of Laboratory Medicine and Pathology, Endocrinology, Internal Medicine, University of Dresden, 01307 Dresden, Germany

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Introduction Phaeochromocytomas and paragangliomas (PPGLs) are heterogeneous tumours with highly variable signs and symptoms and diverse clinical presentations ( Manger 2009 ). Much of the heterogeneity is due to wide-ranging variations in the types

Article Type:
Research Article
DOI:
https://doi.org/10.1677/ERC-10-0211
Volume/Issue:
Volume 18: Issue 1
Free access
Dewi Astuti Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK
Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Christopher J Ricketts Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK
Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Rasheduzzaman Chowdhury Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Michael A McDonough Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Dean Gentle Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK
Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Gail Kirby Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK
Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Susanne Schlisio Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK
Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Rajappa S Kenchappa Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Bruce D Carter Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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William G Kaelin Jr Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Peter J Ratcliffe Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Christopher J Schofield Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Farida Latif Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK
Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Eamonn R Maher Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK
Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK
Centre for Rare Diseases and Personalised Medicine, CRUK Renal Molecular Oncology Group, Chemistry Research Laboratory, Howard Hughes Medical Institute, Oxygen Sensing and Cancer Laboratory, Department of Biochemistry and Center for Molecular Neuroscience, Henry Wellcome Building for Molecular Physiology, West Midlands Regional Genetics Service, University of Birmingham, Birmingham B15 2TT, UK

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Introduction Germline mutations in the von Hippel–Lindau ( VHL ) tumour suppressor gene and in the B, C and D subunits of succinate dehydrogenase ( SDHB , SDHC and SDHD ) are strongly linked with susceptibility to phaeochromocytoma ( Latif et al

Article Type:
Research Article
DOI:
https://doi.org/10.1677/ERC-10-0113
Volume/Issue:
Volume 18: Issue 1
Open access
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C D E Margetts
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D Astuti
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D C Gentle
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W N Cooper
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A Cascon
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D Catchpoole
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M Robledo
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H P H Neumann
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F Latif
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E R Maher
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Introduction Phaeochromocytomas are catecholamine-producing tumours that usually arise within the adrenal medulla but are extra-adrenal in 10% of cases. Hypertension is the most consistent clinical feature of phaeochromocytoma and

Article Type:
Research Article
DOI:
https://doi.org/10.1677/erc.1.00865
Volume/Issue:
Volume 12: Issue 1
Free access
Esther Korpershoek Departments of Pathology, Neurology, Department of Paediatric Oncology and Haematology, Josephine Nefkens Institute, Erasmus Medical Centre, University Medical Centre Rotterdam, PO Box 2040, 3000 CA Rotterdam, The Netherlands

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Nanne K Kloosterhof Departments of Pathology, Neurology, Department of Paediatric Oncology and Haematology, Josephine Nefkens Institute, Erasmus Medical Centre, University Medical Centre Rotterdam, PO Box 2040, 3000 CA Rotterdam, The Netherlands
Departments of Pathology, Neurology, Department of Paediatric Oncology and Haematology, Josephine Nefkens Institute, Erasmus Medical Centre, University Medical Centre Rotterdam, PO Box 2040, 3000 CA Rotterdam, The Netherlands

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Angelique Ziel-van der Made Departments of Pathology, Neurology, Department of Paediatric Oncology and Haematology, Josephine Nefkens Institute, Erasmus Medical Centre, University Medical Centre Rotterdam, PO Box 2040, 3000 CA Rotterdam, The Netherlands

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Hanneke Korsten Departments of Pathology, Neurology, Department of Paediatric Oncology and Haematology, Josephine Nefkens Institute, Erasmus Medical Centre, University Medical Centre Rotterdam, PO Box 2040, 3000 CA Rotterdam, The Netherlands

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Lindsey Oudijk Departments of Pathology, Neurology, Department of Paediatric Oncology and Haematology, Josephine Nefkens Institute, Erasmus Medical Centre, University Medical Centre Rotterdam, PO Box 2040, 3000 CA Rotterdam, The Netherlands

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Jan Trapman Departments of Pathology, Neurology, Department of Paediatric Oncology and Haematology, Josephine Nefkens Institute, Erasmus Medical Centre, University Medical Centre Rotterdam, PO Box 2040, 3000 CA Rotterdam, The Netherlands

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Winand N M Dinjens Departments of Pathology, Neurology, Department of Paediatric Oncology and Haematology, Josephine Nefkens Institute, Erasmus Medical Centre, University Medical Centre Rotterdam, PO Box 2040, 3000 CA Rotterdam, The Netherlands

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Ronald R de Krijger Departments of Pathology, Neurology, Department of Paediatric Oncology and Haematology, Josephine Nefkens Institute, Erasmus Medical Centre, University Medical Centre Rotterdam, PO Box 2040, 3000 CA Rotterdam, The Netherlands

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Introduction In humans, phaeochromocytomas (PCCs) are relatively rare tumours that occur in the adrenal medulla and usually overproduce catecholamines, such as adrenaline or noradrenaline ( Lenders et al . 2005 ). This overproduction causes high

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-12-0088
Volume/Issue:
Volume 19: Issue 6
Free access
Anna Angelousi Department of Pathophysiology, Sector of Endocrinology, National & Kapodistrian University of Athens, Athens, Greece

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Georgios K Dimitriadis Division of Translational and Experimental Medicine, University of Warwick Medical School, Clinical Sciences Research Laboratories, Coventry, UK

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Georgios Zografos Third Department of Surgery, Athens General Hospital “Georgios Gennimatas”, Athens, Greece

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Svenja Nölting Department of Internal Medicine II, Campus Grosshadern, University-Hospital, Ludwig-Maximilians-University of Munich, Munich, Germany

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Gregory Kaltsas Department of Pathophysiology, Sector of Endocrinology, National & Kapodistrian University of Athens, Athens, Greece
Division of Translational and Experimental Medicine, University of Warwick Medical School, Clinical Sciences Research Laboratories, Coventry, UK
Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford, UK

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Ashley Grossman Department of Endocrinology, Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford, UK

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, Lenders et al. 2014 ). Adrenocortical carcinomas (ACCs) account for up to 11% (range 1.2–12%) of adrenocortical tumours, whereas up to 25% of phaeochromocytomas (PCs) may become malignant ( Ayala-Ramirez et al. 2011 , Fassnacht et al. 2016

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-16-0542
Volume/Issue:
Volume 24: Issue 6
Free access
Rui M B Maciel Division of Endocrinology, Department of Medicine, Universidade Federal de São Paulo Escola Paulista de Medicina, São Paulo, São Paulo, Brazil

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Larissa V Bim Division of Genetics, Department of Morphology and Genetics, Universidade Federal de São Paulo Escola Paulista de Medicina, São Paulo, São Paulo, Brazil

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Cleber P Camacho Division of Endocrinology, Department of Medicine, Universidade Federal de São Paulo Escola Paulista de Medicina, São Paulo, São Paulo, Brazil

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Janete M Cerutti Division of Genetics, Department of Morphology and Genetics, Universidade Federal de São Paulo Escola Paulista de Medicina, São Paulo, São Paulo, Brazil

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Dear Editor, In a recent issue of Endocrine-Related Cancer , Castinetti et al . reported a different natural history of phaeochromocytoma in multiple endocrine neoplasia type 2 (MEN 2) patients between Europe and South America ( Castinetti

Article Type:
Letter
DOI:
https://doi.org/10.1530/ERC-17-0349
Volume/Issue:
Volume 25: Issue 9
Free access
Emma R Woodward Section of Medical and Molecular Genetics and Cancer Research UK Renal Molecular Oncology Group, University of Birmingham, Institute of Biomedical Research, Birmingham B15 2TT, UK and West Midlands Regional Genetics Service, Birmingham Women’s Hospital, Birmingham B15 2TG, UK

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Eamonn R Maher Section of Medical and Molecular Genetics and Cancer Research UK Renal Molecular Oncology Group, University of Birmingham, Institute of Biomedical Research, Birmingham B15 2TT, UK and West Midlands Regional Genetics Service, Birmingham Women’s Hospital, Birmingham B15 2TG, UK

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Introduction Although the first descriptions of von Hippel-Lindau (VHL) disease were in the 19th century, phaeochromocytoma was first associated with VHL disease only about 50 years ago ( Glushien et al. 1953 ). Traditionally, an

Article Type:
Review Article
DOI:
https://doi.org/10.1677/erc.1.00683
Volume/Issue:
Volume 13: Issue 2
Free access
Umasuthan Srirangalingam
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Bernard Khoo
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Lisa Walker Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Fiona MacDonald Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Robert H Skelly Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Emad George Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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David Spooner Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Linda B Johnston
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John P Monson
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Ashley B Grossman
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W M Drake
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Scott A Akker
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Patrick J Pollard Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Nick Plowman Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Norbert Avril Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Daniel M Berney Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Jacky M Burrin Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Rodney H Reznek Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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V K Ajith Kumar Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Eamonn R Maher Department of Endocrinology, Department of Clinical Genetics, Department of Medical and Molecular Genetics, Department of Medicine, Department of Medicine, Oncology Centre, Henry Wellcome Building for Molecular Physiology, Radiation Oncology, Center for Cancer Imaging, Cellular Pathology, Clinical Biochemistry, Clinical Genetics, St Bartholomew's Hospital, West Smithfield, London EC1A 7BE, UK

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Shern L Chew
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Introduction Recent studies have shown that ∼25% of apparently sporadic phaeochromocytomas and paragangliomas are due to germ line mutations in one of several familial syndrome genes, including von Hippel-Lindau ( VHL ), RET , succinate

Article Type:
Research Article
DOI:
https://doi.org/10.1677/ERC-08-0239
Volume/Issue:
Volume 16: Issue 2
Free access
Joanna Grey Association for Multiple Endocrine Neoplasia Disorders (AMEND), Tunbridge Wells, Kent, UK

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Kym Winter Association for Multiple Endocrine Neoplasia Disorders (AMEND), Tunbridge Wells, Kent, UK

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MEN3). MEN2 predisposes patients to medullary thyroid cancer (99%) and phaeochromocytoma (50%) but also to other conditions according to specific genotype. MEN2A also includes a 25% risk of developing parathyroid hyperplasia and is now recognised as 4

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-17-0335
Volume/Issue:
Volume 25: Issue 2
Free access