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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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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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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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Dahlia F Davidoff Cancer Genetics, Kolling Institute, Royal North Shore Hospital, St Leonards, New South Wales, Australia
University of Sydney, Camperdown, New South Wales, Australia
Department of Endocrinology, Royal North Shore Hospital, St Leonards, New South Wales, Australia

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Eugénie S Lim Department of Endocrinology, William Harvey Research Institute, Queen Mary University of London, London, UK
Department of Endocrinology, St. Bartholomew’s Hospital, Barts Health NHS Trust, London, UK

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

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Yuvanaa Subramaniam Department of Endocrinology, St. Bartholomew’s Hospital, Barts Health NHS Trust, London, UK

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Eleanor Dorman Department of Endocrinology, St. Bartholomew’s Hospital, Barts Health NHS Trust, London, UK

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John R Burgess Department of Diabetes and Endocrinology, Royal Hobart Hospital, Hobart, Tasmania, Australia
School of Medicine, University of Tasmania, Hobart, Tasmania, Australia

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Scott A Akker Department of Endocrinology, William Harvey Research Institute, Queen Mary University of London, London, UK
Department of Endocrinology, St. Bartholomew’s Hospital, Barts Health NHS Trust, London, UK

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

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Introduction The phaeochromocytoma and paraganglioma (PPGL) tumour group is the most heritable of tumours, with at least 40% of cases arising from a pathogenic germline mutation ( Dahia 2014 ). Of these, around half are caused by pathogenic

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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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E Kim Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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E M Rath Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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V H M Tsang Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia
Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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A P Duff Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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B G Robinson Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia
Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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W B Church Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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D E Benn Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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T Dwight Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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R J Clifton-Bligh Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia
Cancer Genetics, Department of Endocrinology, Faculty of Pharmacy, Australian Nuclear Science and Technology Organisation, Kolling Institute of Medical Research, Royal North Shore Hospital, and University of Sydney, Sydney, New South Wales, Australia

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Introduction Phaeochromocytoma (PC) and paraganglioma (PGL) are rare neuroendocrine tumours that arise from adrenal glands and extra-adrenal sites respectively ( Amar et al . 2005 , Gimenez-Roqueplo et al . 2012 ). These tumours occur in the

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Paul Benjamin Loughrey Patrick G Johnston Centre for Cancer Research, Queen’s University, Belfast, UK
Regional Centre for Endocrinology and Diabetes, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK

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Federico Roncaroli Geoffrey Jefferson Brain Research Centre, Division of Neuroscience and Experimental Psychology, School of Medicine, Manchester University, Manchester, UK

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Estelle Healy Department of Cellular Pathology, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK

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Philip Weir Department of Neurosurgery, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK

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Madhu Basetti Cancer Research UK Cambridge Institute, Li Ka Shing Centre, University of Cambridge, Cambridge, UK

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Ruth T Casey Department of Endocrinology, Cambridge University Hospital NHS Foundation Trust, Cambridge, UK

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Steven J Hunter Regional Centre for Endocrinology and Diabetes, Royal Victoria Hospital, Belfast Health & Social Care Trust, Belfast, UK

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Márta Korbonits Centre for Endocrinology, William Harvey Research Institute, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, London, UK

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Introduction Primary tumours of adenohypophyseal cells recently suggested to be redefined as pituitary neuroendocrine tumours (PitNETs) can rarely occur in association with paraganglioma (PGL) or phaeochromocytoma. These tumours may develop in

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Caroline D E Margetts Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research

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Mark Morris Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research
Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research

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Dewi Astuti Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research
Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research

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Dean C Gentle Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research
Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research

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Alberto Cascon Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research

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Fiona E McRonald Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research
Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research

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Daniel Catchpoole Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research

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Mercedes Robledo Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research

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Hartmut P H Neumann Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research

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Farida Latif Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research
Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research

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Eamonn R Maher Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research
Department of Medical and Molecular Genetics, Cancer Research UK Renal Molecular Oncology Research Group, Hereditary Endocrine Cancer Group, The Tumour Bank, Department of Nephrology, Institute of Biomedical Research

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Introduction Neuroblastoma and phaeochromocytoma are the commonest neural crest-derived tumours in children and adults respectively. Most phaeochromocytomas are benign catecholamine-producing tumours arising within the adrenal medulla, but may be

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Alexandra Chrisoulidou
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Gregory Kaltsas
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Ioannis Ilias
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Ashley B Grossman
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Introduction Phaeochromocytomas are tumours arising from chromaffin tissue of the adrenal medulla, whereas paragangliomas are chromaffin-cell tumours located at extra-adrenal sites along the sympathetic and/or the parasympathetic

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