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Wiebke Werner Department of Hepatology and Gastroenterology, Charité Universitaetsmedizin Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany

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Katharina Detjen Department of Hepatology and Gastroenterology, Charité Universitaetsmedizin Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany

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Alix Bruneau Department of Hepatology and Gastroenterology, Charité Universitaetsmedizin Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany

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Isabella Lurje Department of Hepatology and Gastroenterology, Charité Universitaetsmedizin Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany

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Natalie Nestel Department of Hepatology and Gastroenterology, Charité Universitaetsmedizin Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany

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Henning Jann Department of Hepatology and Gastroenterology, Charité Universitaetsmedizin Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany

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Frank Tacke Department of Hepatology and Gastroenterology, Charité Universitaetsmedizin Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany

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Bertram Wiedenmann Department of Hepatology and Gastroenterology, Charité Universitaetsmedizin Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany

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Christoph Roderburg Department of Hepatology and Gastroenterology, Charité Universitaetsmedizin Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany
Clinic for Gastroenterology, Hepatology and Infectious Diseases, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine, University Düsseldorf, Düsseldorf, Germany

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Linda Hammerich Department of Hepatology and Gastroenterology, Charité Universitaetsmedizin Berlin, Campus Virchow-Klinikum and Campus Charité Mitte, Berlin, Germany

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Introduction Gastroenteropancreatic neuroendocrine neoplasms (GEP-NEN) account for approximately two-thirds of all neuroendocrine malignancies ( Kaliszewski et al. 2022 ). The current World Health Organization (WHO) classification system

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M Heetfeld
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C N Chougnet Department of Hepatology and Gastroenterology, Department of Nuclear Medicine, Department of Surgical Gastroenterology, Department of Internal Medicine, Department of Gastroenterology, Department of Medical Oncology, Department of Medical Oncology, Department of Clinical Medicine and Gastroenterology, Department of Hepatology and Gastroenterology, Charité University Hospital Berlin, Berlin, Germany

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I H Olsen Department of Hepatology and Gastroenterology, Department of Nuclear Medicine, Department of Surgical Gastroenterology, Department of Internal Medicine, Department of Gastroenterology, Department of Medical Oncology, Department of Medical Oncology, Department of Clinical Medicine and Gastroenterology, Department of Hepatology and Gastroenterology, Charité University Hospital Berlin, Berlin, Germany

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A Rinke Department of Hepatology and Gastroenterology, Department of Nuclear Medicine, Department of Surgical Gastroenterology, Department of Internal Medicine, Department of Gastroenterology, Department of Medical Oncology, Department of Medical Oncology, Department of Clinical Medicine and Gastroenterology, Department of Hepatology and Gastroenterology, Charité University Hospital Berlin, Berlin, Germany

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I Borbath Department of Hepatology and Gastroenterology, Department of Nuclear Medicine, Department of Surgical Gastroenterology, Department of Internal Medicine, Department of Gastroenterology, Department of Medical Oncology, Department of Medical Oncology, Department of Clinical Medicine and Gastroenterology, Department of Hepatology and Gastroenterology, Charité University Hospital Berlin, Berlin, Germany

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G Crespo Department of Hepatology and Gastroenterology, Department of Nuclear Medicine, Department of Surgical Gastroenterology, Department of Internal Medicine, Department of Gastroenterology, Department of Medical Oncology, Department of Medical Oncology, Department of Clinical Medicine and Gastroenterology, Department of Hepatology and Gastroenterology, Charité University Hospital Berlin, Berlin, Germany

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J Barriuso Department of Hepatology and Gastroenterology, Department of Nuclear Medicine, Department of Surgical Gastroenterology, Department of Internal Medicine, Department of Gastroenterology, Department of Medical Oncology, Department of Medical Oncology, Department of Clinical Medicine and Gastroenterology, Department of Hepatology and Gastroenterology, Charité University Hospital Berlin, Berlin, Germany

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M Pavel
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D O'Toole Department of Hepatology and Gastroenterology, Department of Nuclear Medicine, Department of Surgical Gastroenterology, Department of Internal Medicine, Department of Gastroenterology, Department of Medical Oncology, Department of Medical Oncology, Department of Clinical Medicine and Gastroenterology, Department of Hepatology and Gastroenterology, Charité University Hospital Berlin, Berlin, Germany

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T Walter Department of Hepatology and Gastroenterology, Department of Nuclear Medicine, Department of Surgical Gastroenterology, Department of Internal Medicine, Department of Gastroenterology, Department of Medical Oncology, Department of Medical Oncology, Department of Clinical Medicine and Gastroenterology, Department of Hepatology and Gastroenterology, Charité University Hospital Berlin, Berlin, Germany

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other Knowledge Network members
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Introduction Gastroenteropancreatic neuroendocrine neoplasms (GEP-NEN) are rare tumors defined by the presence of specific biomarkers ( Yao et al . 2008 ). The differentiation status is a major prognostic factor, regardless of primary site and

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Vincenzo Marotta Department of Clinical Medicine and Surgery, Federico II University, Naples, Italy

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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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Concetta Sciammarella IOS & COLEMAN Srl, Naples, Italy

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Maria Rosaria Ambrosio Section of Endocrinology and Internal Medicine, Department of Medical Sciences, University of Ferrara, Ferrara, Italy

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Marta Bondanelli Section of Endocrinology and Internal Medicine, Department of Medical Sciences, University of Ferrara, Ferrara, Italy

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Annamaria Colao Department of Clinical Medicine and Surgery, Federico II University, Naples, Italy

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Antongiulio Faggiano Thyroid and Parathyroid Surgery Unit, Istituto Nazionale per lo Studio e la Cura dei Tumori ‘Fondazione G. Pascale’ – IRCCS, Naples, Italy

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Introduction Despite being rare diseases, neuroendocrine neoplasms (NENs) have shown a worldwide increase in the past several decades, with incidence rates rising from 1.52 to 7.41 cases per 100,000 from 1973 to 2012 ( Leoncini et al . 2017

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Sebastian Krug Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Benjamin Kühnemuth Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Heidi Griesmann Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Albrecht Neesse Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Leonie Mühlberg Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Michael Boch Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Juliane Kortenhaus Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Volker Fendrich Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Dominik Wiese Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Bence Sipos Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Juliane Friemel Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Thomas M Gress Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Patrick Michl Departments of Gastroenterology, Surgery, Department of Pathology, Department of Pathology, Endocrinology and Metabolism

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Introduction Gastroenteropancreatic neuroendocrine neoplasms (GEP–NENs) represent a heterogeneous group of solid cancers, on the basis of on embryogenic, morphological and biochemical findings ( Modlin et al . 2008 , Rindi & Wiedenmann 2012 ). The

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Kenzo Nakano
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Toshihiko Masui Department of Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan

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Akitada Yogo Department of Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan

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Yuichiro Uchida Department of Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan

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Asahi Sato Department of Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan

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Yosuke Kasai Department of Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan

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Kazuyuki Nagai Department of Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan

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Takayuki Anazawa
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Yoshiya Kawaguchi Department of Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan

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Shinji Uemoto Department of Surgery, Kyoto University Graduate School of Medicine, Kyoto, Japan

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Introduction Pancreatic neuroendocrine neoplasms (PanNENs) comprise approximately 1–2% of all pancreatic neoplasms ( Yao et al. 2008 ). The 2017 World Health Organization (WHO) classification divides PanNENs into well

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Julia Beck ENETS Center of Excellence for Neuroendocrine and Endocrine Tumors, University Hospital Basel, Basel, Switzerland
Department of Clinical Research, University of Basel, Basel, Switzerland

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Alexander Siebenhüner Hirslanden Zurich AG, Clinic for Hematology and Oncology, Zurich, Switzerland

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Damian Wild ENETS Center of Excellence for Neuroendocrine and Endocrine Tumors, University Hospital Basel, Basel, Switzerland
Division of Nuclear Medicine, University Hospital Basel, Basel, Switzerland

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Emanuel Christ ENETS Center of Excellence for Neuroendocrine and Endocrine Tumors, University Hospital Basel, Basel, Switzerland
Department of Clinical Research, University of Basel, Basel, Switzerland

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Julie Refardt ENETS Center of Excellence for Neuroendocrine and Endocrine Tumors, University Hospital Basel, Basel, Switzerland
Department of Clinical Research, University of Basel, Basel, Switzerland

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little information about the impact of sex differences are neuroendocrine neoplasms (NENs). NENs usually are well-differentiated tumors, mainly localized in the gastrointestinal system, pancreas, and lung ( Klöppel 2011 , Caplin et al. 2015 , Refardt

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Lena Weindl Department of Medicine 1, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany

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Imke Atreya Department of Medicine 1, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany

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Peter Dietrich Department of Medicine 1, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany
Institute of Biochemistry, Emil-Fischer-Zentrum, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany

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Sabine Neubeck Department of Medicine 1, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany

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Markus F Neurath Department of Medicine 1, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany
Deutsches Zentrum Immuntherapie DZI, Erlangen, Germany
Comprehensive Cancer Center CCC-EMN, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany

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Marianne Pavel Department of Medicine 1, University Hospital Erlangen, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany
Deutsches Zentrum Immuntherapie DZI, Erlangen, Germany
Comprehensive Cancer Center CCC-EMN, Friedrich-Alexander University Erlangen-Nürnberg, Erlangen, Germany

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Introduction Neuroendocrine neoplasms (NENs) represent a rare and heterogeneous group of malignancies with variable tumor growth ( Pavel & Sers 2016 ). They occur with an estimated incidence of 6.98 cases/100,000 citizens ( Dasari et al

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Alexa Childs UCL Cancer Institute, University College London, London, UK

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Christopher D Steele UCL Cancer Institute, University College London, London, UK

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Clare Vesely UCL Cancer Institute, University College London, London, UK

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Francesca M Rizzo UCL Cancer Institute, University College London, London, UK

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Leah Ensell UCL Cancer Institute, University College London, London, UK

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Helen Lowe UCL Cancer Institute, University College London, London, UK

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Pawan Dhami UCL Cancer Institute, University College London, London, UK

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Heli Vaikkinen UCL Cancer Institute, University College London, London, UK

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Tu Vinh Luong Department of Histopathology, Royal Free London NHS Foundation Trust, London, UK

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Lucia Conde UCL Cancer Institute, University College London, London, UK

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Javier Herrero UCL Cancer Institute, University College London, London, UK

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Martyn Caplin Department of Gastroenterology, Royal Free London NHS Foundation Trust, London, UK

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Christos Toumpanakis Department of Gastroenterology, Royal Free London NHS Foundation Trust, London, UK

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Christina Thirlwell UCL Cancer Institute, University College London, London, UK
Department of Oncology, Royal Free London NHS Foundation Trust, London, UK

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John A Hartley UCL Cancer Institute, University College London, London, UK

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Nischalan Pillay Research Department of Pathology, Cancer Institute, University College London, London, UK
Department of Cellular and Molecular Pathology, Royal National Orthopaedic Hospital NHS Trust, Stanmore, Middlesex, UK

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Tim Meyer UCL Cancer Institute, University College London, London, UK
Department of Oncology, Royal Free London NHS Foundation Trust, London, UK

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. 2011 , Poveda et al. 2011 ), including neuroendocrine neoplasms ( Khan et al. 2011 , 2013 , 2016 , Mandair et al. 2021 ). More recently, molecular analysis of single CTCs has been used to identify predictive biomarkers, such as the T790M

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Federica Grillo Pathology Unit, Department of Surgical Sciences and Integrated Diagnostics (DISC), University of Genova, Genova, Italy
Ospedale Policlinico San Martino IRCCS, Genova, Italy

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Tullio Florio Pharmacology Unit, Department of Internal Medicine (DIMI), University of Genova, Genova, Italy

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Francesco Ferraù Department of Human Pathology of Adulthood and Childhood, University of Messina, Messina, Italy

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Elda Kara Unit of Endocrinology, Metabolism, Diabetology and Nutrition, Azienda Sanitaria Universitaria Integrata di Udine, Ospedale Santa Maria della Misericordia, Udine, Italy

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Giuseppe Fanciulli Neuroendocrine Tumours Unit, Department of Clinical and Experimental Medicine, University of Sassari – AOU Sassari, Sassari, Italy

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Antongiulio Faggiano Department of Clinical Medicine and Surgery, University ‘Federico II’, Naples, Italy

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Annamaria Colao Department of Clinical Medicine and Surgery, University ‘Federico II’, Naples, Italy

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NIKE Group
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Introduction Neuroendocrine neoplasms (NENs) are a heterogeneous family of neoplasms originating from neuroendocrine cells dispersed in different organs, most frequently in the digestive system and in the lungs. The last few decades have

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Julie Refardt ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, Erasmus Medical Center and Erasmus Cancer Institute, Rotterdam, The Netherlands
ENETS Center of Excellence, Department of Endocrinology, University Hospital Basel, Basel, Switzerland

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Caroline M den Hoed Department of Gastroenterology and Hepatology, Erasmus MC Transplant Institute, Erasmus MC University Medical Center, Rotterdam, The Netherlands

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Janneke Langendonk Department of Internal Medicine, Center for Lysosomal and Metabolic Diseases, Erasmus Medical Center, Rotterdam, The Netherlands

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Wouter T Zandee Division of Endocrinology, Department of Internal Medicine, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands

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Ayoub Charehbili ENETS Center of Excellence, Department of Radiology & Nuclear Medicine, Erasmus Medical Center, Rotterdam, The Netherlands

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Richard A Feelders ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, Erasmus Medical Center and Erasmus Cancer Institute, Rotterdam, The Netherlands

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Wouter W de Herder ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, Erasmus Medical Center and Erasmus Cancer Institute, Rotterdam, The Netherlands

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Tessa Brabander ENETS Center of Excellence, Department of Radiology & Nuclear Medicine, Erasmus Medical Center, Rotterdam, The Netherlands

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Johannes Hofland ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, Erasmus Medical Center and Erasmus Cancer Institute, Rotterdam, The Netherlands

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overproduction or due to infections with urease-producing bacteria, for example, urinary tract or skin infections ( Stergachis et al. 2020 ). Ten cases of hyperammonemia in neuroendocrine neoplasm (NEN) patients have been described in the literature to date

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