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Elham Barazeghi Department of Surgical Sciences, Uppsala University, Uppsala University Hospital, Rudbeck Laboratory, Uppsala, Sweden

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Per Hellman Department of Surgical Sciences, Uppsala University, Uppsala University Hospital, Rudbeck Laboratory, Uppsala, Sweden

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Gunnar Westin Department of Surgical Sciences, Uppsala University, Uppsala University Hospital, Rudbeck Laboratory, Uppsala, Sweden

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Peter Stålberg Department of Surgical Sciences, Uppsala University, Uppsala University Hospital, Rudbeck Laboratory, Uppsala, Sweden

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give rise to carcinoid syndrome as a major cause of morbidity and mortality in approximately 20% of SI-NET patients. To date, genetic and epigenetic alterations in SI-NETs are poorly studied. Massive DNA sequencing analysis on SI-NETs revealed that

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Elena Pardi Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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Stefano Mariotti Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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Natalia S Pellegata Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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Katiuscia Benfini Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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Simona Borsari Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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Federica Saponaro Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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Liborio Torregrossa Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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Antonello Cappai Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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Chiara Satta Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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Marco Mastinu Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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Claudio Marcocci Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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Filomena Cetani Endocrine Unit 2, Endocrinology Unit, Institute of Pathology, Department of Surgical, Department of Clinical and Experimental Medicine, University Hospital of Pisa, University of Pisa, Via Paradisa 2, Pisa, Italy

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pituitary tumors in the same individual (1) . A minority of affected patients may also develop a wide spectrum of endocrine and non-endocrine manifestations, such as adrenal cortical tumors, foregut carcinoid tumors, angiofibromas, collagenomas, and lipomas

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Anela Blažević Department of Internal Medicine, Section Endocrinology, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands

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Anand M Iyer Department of Internal Medicine, Section Endocrinology, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands

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Marie-Louise F van Velthuysen Department of Pathology, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands

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Johannes Hofland Department of Internal Medicine, Section Endocrinology, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands

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Peter M van Koestveld Department of Internal Medicine, Section Endocrinology, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands

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Gaston J H Franssen Department of Surgery, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands

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Richard A Feelders Department of Internal Medicine, Section Endocrinology, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands

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Marina Zajec Laboratory of Neuro-Oncology/Clinical & Cancer Proteomics, Department of Neurology, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands

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Theo M Luider Laboratory of Neuro-Oncology/Clinical & Cancer Proteomics, Department of Neurology, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands

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Wouter W de Herder Department of Internal Medicine, Section Endocrinology, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands

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Leo J Hofland Department of Internal Medicine, Section Endocrinology, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, Netherlands

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Introduction Small intestinal neuroendocrine tumors (SI-NETs) are accompanied by specific clinical pathology, most notable carcinoid syndrome and fibrotic complications such as carcinoid heart disease and mesenteric fibrosis (MF) ( 1 ). There

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Magaly Zappa Univ Paris Diderot, Sorbonne Paris Cité, Paris, France
INSERM, UMR1149 CRI, Paris, France
Department of Radiology, AP-HP, Clichy, France

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Olivia Hentic Department of Gastroenterology and Pancreatology, AP-HP, Clichy, France

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Marie-Pierre Vullierme Department of Radiology, AP-HP, Clichy, France

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Matthieu Lagadec Department of Radiology, AP-HP, Clichy, France

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Maxime Ronot Univ Paris Diderot, Sorbonne Paris Cité, Paris, France
INSERM, UMR1149 CRI, Paris, France
Department of Radiology, AP-HP, Clichy, France

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Philippe Ruszniewski Univ Paris Diderot, Sorbonne Paris Cité, Paris, France
INSERM, UMR1149 CRI, Paris, France
Department of Gastroenterology and Pancreatology, AP-HP, Clichy, France

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Valérie Vilgrain Univ Paris Diderot, Sorbonne Paris Cité, Paris, France
INSERM, UMR1149 CRI, Paris, France
Department of Radiology, AP-HP, Clichy, France

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Theodorsson E Wilander E Oberg K. Carcinoid tumors: analysis of prognostic factors and survival in 301 patients from a referral center . Annals of Oncology 1997 8 685 – 690 . ( doi:10.1023/A:1008215730767 ) 18 Turner GB Johnston BT

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Ulrik Ø Andersen Department of Clinical Biochemistry, Rigshospitalet, Copenhagen, Denmark
Institute of Biomedical Sciences, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark

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Dijana Terzic Department of Clinical Biochemistry, Rigshospitalet, Copenhagen, Denmark
Institute of Biomedical Sciences, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark

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Nicolai Jacob Wewer Albrechtsen Department of Clinical Biochemistry, Rigshospitalet, Copenhagen, Denmark
Institute of Biomedical Sciences, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark
Novo Nordisk Foundation Centre for Protein Research, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark

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Peter Dall Mark Department of Clinical Biochemistry, Rigshospitalet, Copenhagen, Denmark
Institute of Biomedical Sciences, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark

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Peter Plomgaard Department of Clinical Biochemistry, Rigshospitalet, Copenhagen, Denmark
Department of Clinical Medicine, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark

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Jens F Rehfeld Department of Clinical Biochemistry, Rigshospitalet, Copenhagen, Denmark

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Finn Gustafsson Department of Cardiology, Rigshospitalet, Copenhagen, Denmark

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Jens P Goetze Department of Clinical Biochemistry, Rigshospitalet, Copenhagen, Denmark
Institute of Biomedical Sciences, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark

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hypergastrinemia of antral origin . American Journal of Pathology 1978 93 53 – 68 . 21 Havu N Mattsson H Ekman L Carlsson E . Enterochromaffin-like cell carcinoids in the rat gastric mucosa following long-term administration of ranitidine

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Anna Malczewska Department of Endocrinology and Neuroendocrine Tumours, Medical University of Silesia, Katowice, Poland

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Kjell Oberg Department of Endocrine Oncology, University Hospital, Uppsala, Sweden

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Beata Kos-Kudla Department of Endocrinology and Neuroendocrine Tumours, Medical University of Silesia, Katowice, Poland

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; duodenal, DNENs, n = 9, and bronchopulmonary carcinoids (BPC, n = 43). The cohort was grouped into image-positive disease (IPD, n = 123), and image-negative disease (IND, n = 135) based on evidence vs no evidence of disease on anatomical and

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Sara Storvall Department of Endocrinology, Abdominal Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Helena Leijon Department of Pathology and Huslab, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Eeva Ryhänen Department of Endocrinology, Abdominal Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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Johanna Louhimo Department of Surgery, 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

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Camilla Schalin-Jäntti Department of Endocrinology, Abdominal Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland

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

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previously used and verified ( 25 , 26 , 27 , 28 , 33 ) with Western blotting and/or in vitro somatostatin receptor autoradiography. The same antibodies have previously been used by our research group for a study on pulmonary carcinoid tumors ( 23 ) as

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Peiwen Wu Department of Radiation Oncology, The First Affiliated Hospital of Xi’an Jiaotong University, Xi’an, China

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Dongjie He Department of Radiation Oncology, Tangdu Hospital, the Second Affiliated Hospital of Air Force Medical University, Xi’an, China

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Hao Chang Department of Radiation Oncology, Tangdu Hospital, the Second Affiliated Hospital of Air Force Medical University, Xi’an, China

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Xiaozhi Zhang Department of Radiation Oncology, The First Affiliated Hospital of Xi’an Jiaotong University, Xi’an, China

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JN Rashid A , et al . One hundred years after ‘carcinoid’: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States . Journal of Clinical Oncology 2008 26 3063 – 3072 . ( https://doi.org/10.1200/JCO

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Anna Malczewska Department of Endocrinology and Neuroendocrine Tumors, Department of Pathophysiology and Endocrinology, Medical University of Silesia, Katowice, Poland

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Magdalena Witkowska Department of Endocrinology and Neuroendocrine Tumors, Department of Pathophysiology and Endocrinology, Medical University of Silesia, Katowice, Poland

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Karolina Makulik Department of Endocrinology and Neuroendocrine Tumors, Department of Pathophysiology and Endocrinology, Medical University of Silesia, Katowice, Poland

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Agnes Bocian Department of Endocrinology and Neuroendocrine Tumors, Department of Pathophysiology and Endocrinology, Medical University of Silesia, Katowice, Poland

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Agata Walter Department of Endocrinology and Neuroendocrine Tumors, Department of Pathophysiology and Endocrinology, Medical University of Silesia, Katowice, Poland

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Joanna Pilch-Kowalczyk Department of Radiology and Nuclear Medicine, Medical University of Silesia, Katowice, Poland

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Wojciech Zajęcki Department of Pathology in Zabrze, Medical University of Silesia, Katowice, Poland

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Lisa Bodei Molecular Imaging and Therapy Service, Department of Radiology, Memorial Sloan Kettering Cancer Center, New York, New York, USA

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Kjell Oberg Department of Endocrine Oncology, University Hospital, Uppsala, Sweden

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Beata Kos-Kudła Department of Endocrinology and Neuroendocrine Tumors, Department of Pathophysiology and Endocrinology, Medical University of Silesia, Katowice, Poland

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PNETs without liver involvement, carcinoid syndrome occurs very rarely ( 2 ). Circulating indicators of tumor secretory functionality (amines or peptides) hence represent a minority of NETs. Measurement of secretory biomarkers is therefore de facto

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Ruth Therese Casey Department of Endocrinology, University of Cambridge, Cambridge Biomedical Research Centre, Addenbrooke’s Hospital, Cambridge, UK

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Deborah Saunders East Anglian Regional Radiation Protection Service, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK

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Benjamin George Challis Department of Endocrinology, University of Cambridge, Cambridge Biomedical Research Centre, Addenbrooke’s Hospital, Cambridge, UK

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Deborah Pitfield Department of Endocrinology, University of Cambridge, Cambridge Biomedical Research Centre, Addenbrooke’s Hospital, Cambridge, UK

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Heok Cheow Department of Radiology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK

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Ashley Shaw Department of Radiology, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK

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Helen Lisa Simpson Wolfson Diabetes and Endocrine Clinic, Institute of Metabolic Science, Addenbrooke’s Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK

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associated with hormone hypersecretion due to improved surveillance and timely initiation of treatment. Currently, malignant pancreatic NETs and thymic carcinoid tumours are recognised as the most common causes of death in MEN1, respectively. Recent data

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