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Samira M Sadowski Thoracic and Endocrine Surgery, University Hospitals of Geneva, Geneva, Switzerland

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Emanuel Christ Department of Endocrinology, Diabetes and Metabolism, University Hospital of Basel, Basel, Switzerland

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Benoit Bédat Thoracic and Endocrine Surgery, University Hospitals of Geneva, Geneva, Switzerland

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Attila Kollár Department of Medical Oncology, Inselspital, University of Bern, Bern, Switzerland

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Wolfram Karenovics Thoracic and Endocrine Surgery, University Hospitals of Geneva, Geneva, Switzerland

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Aurel Perren Institute of Pathology, University of Bern, Bern, Switzerland

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Frédéric Triponez Thoracic and Endocrine Surgery, University Hospitals of Geneva, Geneva, Switzerland

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on behalf of the SwissNET registry
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metastasizes locally to hilar lymph nodes, and also distally to other organs ( 5 ). Regarding the management of pulmonary carcinoids, Caplin and coworkers ( 6 ) have published a recent European Neuroendocrine Tumor Society expert consensus with

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Tiina Vesterinen HUSLAB, Department of Pathology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
Institute for Molecular Medicine Finland (FIMM), Helsinki Institute of Life Science, University of Helsinki, Helsinki, Finland

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Teijo Kuopio Department of Biological and Environmental Science, University of Jyväskylä and Department of Pathology, Central Finland Health Care District, Jyväskylä, Finland

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Maarit Ahtiainen Department of Education and Research, Central Finland Central Hospital, Jyväskylä, Finland

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Aija Knuuttila Department of Pulmonary Medicine, Heart and Lung Center, and Cancer Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Harri Mustonen Department of Surgery, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Kaisa Salmenkivi HUSLAB, Department of Pathology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Johanna Arola HUSLAB, Department of Pathology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Caj Haglund Department of Surgery, University of Helsinki and Helsinki University Hospital, Helsinki, Finland
Translational Cancer Medicine Program, Faculty of Medicine, University of Helsinki, Helsinki, Finland

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Introduction Pulmonary carcinoid (PC) tumors are low- and intermediate-grade neoplasms that are subdivided into typical carcinoid (TC) and atypical carcinoid (AC) based on the mitotic count and presence of necrosis ( 1 ). PCs belong to

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Richard P Steeds Department of Cardiology, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK
Institute of Cardiovascular Sciences, University of Birmingham, Birmingham, UK

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Vandana Sagar Centre for Liver and Gastrointestinal Research, Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK

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Shishir Shetty Centre for Liver and Gastrointestinal Research, Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK

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Tessa Oelofse Departments of Anaesthesia and Intensive Care, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK

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Harjot Singh Departments of Anaesthesia and Intensive Care, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK

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Raheel Ahmad Department of Cardiology, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK

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Elizabeth Bradley Therapy Services (Dietetics), University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK

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Rachel Moore Departments of Anaesthesia and Intensive Care, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK

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Suzanne Vickrage Birmingham Neuroendocrine Tumour Centre, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK

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Stacey Smith Birmingham Neuroendocrine Tumour Centre, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK

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Ivan Yim Department of Cardiothoracic Surgery, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK

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Yasir S Elhassan Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK
Institute of Metabolism and Systems Research, University of Birmingham, Birmingham, UK

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Hema Venkataraman Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK

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John Ayuk Centre for Endocrinology, Diabetes and Metabolism, Birmingham Health Partners, Birmingham, UK

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Stephen Rooney Department of Cardiothoracic Surgery, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK

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Tahir Shah Birmingham Neuroendocrine Tumour Centre, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK
Department of Hepatology and Liver Transplantation, University Hospitals Birmingham (Queen Elizabeth), NHS Hospitals Foundation Trust, Birmingham, UK

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Bhabra MS Steeds RP . Transcatheter pulmonary and tricuspid valve-in-valve replacement for bioprosthesis degeneration in carcinoid heart disease . European Heart Journal Cardiovascular Imaging 2016 114 . ( https://doi.org/10.1093/ehjci/jev279

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Filippo Ceccato Endocrinology Unit, Department of Medicine DIMED, University-Hospital of Padova, Padova, Italy
Department of Neuroscience DNS, University of Padova, Padova, Italy

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Diego Cecchin Department of Neuroscience DNS, University of Padova, Padova, Italy
Nuclear Medicine Unit, Department of Medicine – DIMED, University-Hospital of Padova, Padova, Italy
Padova Neuroscience Center PNC, University of Padova, Padova, Italy

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Michele Gregianin Nuclear Medicine Unit, Castelfranco Veneto, Italy

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Giacomo Ricci Department of Neuroscience DNS, University of Padova, Padova, Italy

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Cristina Campi Padova Neuroscience Center PNC, University of Padova, Padova, Italy
Department of Mathematics ‘Tullio Levi-Civita’ DM, University of Padova, Padova, Italy

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Filippo Crimì Radiology Unit, Department of Medicine DIMED, University-Hospital of Padova, Padova, Italy

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Marta Bergamo Endocrinology Unit, Department of Medicine DIMED, University-Hospital of Padova, Padova, Italy

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Annibale Versari Nuclear Medicine Unit, Reggio Emilia, Italy

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Carmelo Lacognata Radiology Department, University-Hospital of Padova, Padova, Italy

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Federico Rea Thoracic Surgery Unit, Department of Cardiac, Thoracic and Vascular Sciences, University Hospital of Padova, Padova, Italy

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Mattia Barbot Endocrinology Unit, Department of Medicine DIMED, University-Hospital of Padova, Padova, Italy

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Carla Scaroni Endocrinology Unit, Department of Medicine DIMED, University-Hospital of Padova, Padova, Italy

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despite a meticulous and extended follow-up ( 4 , 7 ). The most common tumours in EAS are thoracic (lung or mediastinal carcinoids, small cell-lung carcinoma SCLC, thymic tumours and medullary thyroid carcinomas) or abdominal neoplasms (islet cell tumours

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B C M Hermans Department of Pulmonary Diseases, GROW School for Oncology & Developmental Biology, Maastricht University Medical Centre, Maastricht, The Netherlands

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J L Derks Department of Pulmonary Diseases, GROW School for Oncology & Developmental Biology, Maastricht University Medical Centre, Maastricht, The Netherlands

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H J M Groen Department of Pulmonary Diseases, University of Groningen and University Medical Centre, Groningen, The Netherlands

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J A Stigt Department of Pulmonary Diseases, Isala Hospital, Zwolle, The Netherlands

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R J van Suylen Pathology-DNA, Jeroen Bosch Hospital, ‘s Hertogenbosch, The Netherlands

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L M Hillen Department of Pathology, GROW School for Oncology & Developmental Biology, Maastricht University Medical Centre, Maastricht, The Netherlands

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E C van den Broek PALGA Foundation, Utrecht, The Netherlands

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E J M Speel Department of Pathology, GROW School for Oncology & Developmental Biology, Maastricht University Medical Centre, Maastricht, The Netherlands

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A-M C Dingemans Department of Pulmonary Diseases, GROW School for Oncology & Developmental Biology, Maastricht University Medical Centre, Maastricht, The Netherlands

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alterations between atypical carcinoid and LCNEC for TP53 , RB1 and MEN1 ( 7 , 9 ). Figure 1 Ki-67 proliferation indices (PIs) in the spectrum of pulmonary neuroendocrine neoplasms. Carcinoids have a Ki-67 PI ≤20%, whereas LCNEC and SCLC generally

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Katherine Van Loon
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Li Zhang
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Jennifer Keiser
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Cendy Carrasco
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Katherine Glass The UCSF Helen Diller Family Comprehensive Cancer Center, The Ohio State University Comprehensive Cancer Center, National Comprehensive Cancer Network, University of California, San Francisco, 1600 Divisadero Street, UCSF Box 1770, San Francisco, California 94143, USA

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Maria-Teresa Ramirez The UCSF Helen Diller Family Comprehensive Cancer Center, The Ohio State University Comprehensive Cancer Center, National Comprehensive Cancer Network, University of California, San Francisco, 1600 Divisadero Street, UCSF Box 1770, San Francisco, California 94143, USA

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Sarah Bobiak The UCSF Helen Diller Family Comprehensive Cancer Center, The Ohio State University Comprehensive Cancer Center, National Comprehensive Cancer Network, University of California, San Francisco, 1600 Divisadero Street, UCSF Box 1770, San Francisco, California 94143, USA

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Eric K Nakakura
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Alan P Venook
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Manisha H Shah The UCSF Helen Diller Family Comprehensive Cancer Center, The Ohio State University Comprehensive Cancer Center, National Comprehensive Cancer Network, University of California, San Francisco, 1600 Divisadero Street, UCSF Box 1770, San Francisco, California 94143, USA

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Emily K Bergsland
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. Patients were required to have a second visit within 6 months of initial presentation for inclusion in the database. The following rare tumor types were excluded: poorly differentiated pulmonary tumors, high-grade pulmonary or bronchial carcinoids, tumors

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Majunath R Goroshi Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Swati S Jadhav Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Anurag R Lila Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Rajeev Kasaliwal Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Shruti Khare Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Chaitanya G Yerawar Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Priya Hira Department of Radiology, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Uday Phadke Ruby Hall Clinic, Pune, Maharashtra, India

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Hina Shah Department of Nuclear Medicine and Positron Emission Tomography/Computed Tomography, Jaslok Hospital and Research Centre, Mumbai, Maharashtra, India

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Vikram R Lele Department of Nuclear Medicine and Positron Emission Tomography/Computed Tomography, Jaslok Hospital and Research Centre, Mumbai, Maharashtra, India

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Gaurav Malhotra Radiation Medicine Centre, Bhabha Atomic Research Centre, Tata Memorial Centre, Annexe, Parel, Mumbai, Maharashtra, India

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Tushar Bandgar Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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Nalini S Shah Department of Endocrinology, Seth G S Medical College and KEM Hospital, Mumbai, Maharashtra, India

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# 0 3 (lung nodules)** 0 0 107 53 NA – Bilateral adrenalectomy ACTH, adrenocorticotrophic hormone; LDDS, low-dose dexamethasone suppression; BPC, bronchopulmonary carcinoid; DIPNECH, diffuse idiopathic pulmonary neuroendocrine

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David C Llewellyn Department of Endocrinology ASO/EASO COM, King’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK

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Rajaventhan Srirajaskanthan Neuroendocrine Tumour Unit, Kings Health Partners ENETS Centre of Excellence, Denmark Hill, London, UK
Faculty of Life Sciences and Medicine, Kings College London, London, UK

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Royce P Vincent Faculty of Life Sciences and Medicine, Kings College London, London, UK
Department of Clinical Biochemistry, King’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK

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Catherine Guy Department of Cellular Pathology, Royal Sussex County Hospital, Eastern Road, Brighton, UK

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Eftychia E Drakou Department of Clinical Oncology, Guy’s Cancer Centre – Guy’s and St Thomas’ NHS Foundation Trust, Great Maze Pond, London, UK

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Simon J B Aylwin Department of Endocrinology ASO/EASO COM, King’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK
Neuroendocrine Tumour Unit, Kings Health Partners ENETS Centre of Excellence, Denmark Hill, London, UK

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Ashley B Grossman Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK
Barts and the London School of Medicine, Centre for Endocrinology, William Harvey Institute, London, UK
Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK

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John K Ramage Neuroendocrine Tumour Unit, Kings Health Partners ENETS Centre of Excellence, Denmark Hill, London, UK
Faculty of Life Sciences and Medicine, Kings College London, London, UK

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Georgios K Dimitriadis Department of Endocrinology ASO/EASO COM, King’s College Hospital NHS Foundation Trust, Denmark Hill, London, UK
Faculty of Life Sciences and Medicine, School of Life Course Sciences, Obesity Immunometabolism and Diabetes Group, King’s College London, London, UK

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, parathyroid glands, larynx, oesophagus, thymus, lung, small intestine, liver, bladder and adrenals, all of which can resemble MTC histologically ( 3 , 5 , 10 , 12 ). Around 1% of normal pulmonary tissue is made up of neuroendocrine cells, which can be

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M S Elston Department of Endocrinology, Waikato Hospital, Hamilton, New Zealand
Waikato Clinical Campus, University of Auckland, Hamilton, New Zealand

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V B Crawford Department of Endocrinology, Waikato Hospital, Hamilton, New Zealand

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M Swarbrick Department of Radiology, Waikato Hospital, Hamilton, New Zealand

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M S Dray Department of Pathology, Waikato Hospital, Hamilton, New Zealand

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M Head Department of Oncology, Tauranga Hospital, Tauranga, New Zealand

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J V Conaglen Waikato Clinical Campus, University of Auckland, Hamilton, New Zealand

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with EAS. The most common are those of neuroendocrine origin, namely small-cell lung carcinoma (3.3–50%), bronchial carcinoid (4.8–38.9%), thymic carcinoid (4.7–10.6%), medullary thyroid carcinoma (MTC) (1.9–11.6%), gastroenteropancreatic neuroendocrine

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Giulia Bresciani Section of Endocrinology and Internal Medicine, Department of Medical Sciences, University of Ferrara, Ferrara, Italy

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Angeliki Ditsiou Department of Biochemistry and Biomedicine, School of Life Sciences, University of Sussex, Brighton, UK

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Chiara Cilibrasi Department of Biochemistry and Biomedicine, School of Life Sciences, University of Sussex, Brighton, UK

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Viviana Vella Department of Biochemistry and Biomedicine, School of Life Sciences, University of Sussex, Brighton, UK

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Federico Rea Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy

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Marco Schiavon Department of Cardiac, Thoracic and Vascular Sciences, University of Padua, Padua, Italy

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Narciso Giorgio Cavallesco Department of Morphology, Surgery and Experimental Medicine, University of Ferrara, Ferrara, Italy

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Georgios Giamas Department of Biochemistry and Biomedicine, School of Life Sciences, University of Sussex, Brighton, UK

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Maria Chiara Zatelli Section of Endocrinology and Internal Medicine, Department of Medical Sciences, University of Ferrara, Ferrara, Italy

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Teresa Gagliano Department of Biochemistry and Biomedicine, School of Life Sciences, University of Sussex, Brighton, UK

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malignancies originating from different parts of the human body. More than one half (about 70%) occurs in the gastrointestinal tract, while the other 25% occurs mostly in the broncho-pulmonary system ( 3 , 4 ). NENs that affect the bronchial tree are called

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