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M C F Mulders Department of Internal Medicine, ENETS Center of Excellence, Section of Endocrinology, Erasmus Medical Center and Erasmus Medical Center Cancer Institute, Rotterdam, The Netherlands

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Q G de Lussanet de la Sablonière Department of Radiology and Nuclear Medicine, Erasmus Medical Center, Rotterdam, The Netherlands

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M L F van Velthuysen Department of Pathology, Erasmus Medical Center, Rotterdam, The Netherlands

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E M Roes Department of Gynecologic Oncology, Erasmus Medical Center Cancer Institute, Rotterdam, The Netherlands

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

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

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Introduction Neuroendocrine tumors (NETs) are a heterogeneous group of neoplasms that predominantly arise from neuroendocrine cells of the embryonic gut and are subdivided into foregut, midgut and hindgut NETs ( Hofland et al. 2020 ). NET

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Alexa Childs Department of Oncology, Royal Free London NHS Foundation Trust, London, UK

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Amy Kirkwood Cancer Research UK & UCL Cancer Trials Centre, London, UK

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Julien Edeline UCL Cancer Institute, UCL, London, UK

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

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Jennifer Watkins Department of Pathology, Royal Free London NHS Foundation Trust, London, UK

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Angela Lamarca Institute of Cancer Sciences, University of Manchester/The Christie NHS Foundation Trust, Manchester, UK

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Doraid Alrifai King’s College Hospital NHS Foundation Trust, London, UK

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Phyllis Nsiah-Sarbeng Department of Radiology, Royal Free London NHS Foundation Trust, London, UK

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Roopinder Gillmore Department of Oncology, Royal Free London NHS Foundation Trust, London, UK

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Astrid Mayer Department of Oncology, Royal Free London NHS Foundation Trust, London, UK

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

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Debashis Sarker King’s College Hospital NHS Foundation Trust, London, UK

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Juan W Valle Institute of Cancer Sciences, University of Manchester/The Christie NHS Foundation Trust, Manchester, UK

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

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count as prognostic markers for metastatic pancreatic and midgut neuroendocrine neoplasms . British Journal of Cancer 108 1838 – 1845 . ( doi:10.1038/bjc.2013.156 ) Khan MS Kirkwood AA Tsigani T Lowe H Goldstein R Hartley JA

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

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Wouter T Zandee ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, Erasmus Medical Center, 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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Richard A Feelders ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, Erasmus Medical Center, Rotterdam, The Netherlands

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Gaston J H Franssen Department of Surgery, Section of Surgical Oncology, Erasmus Medical Center, Rotterdam, The Netherlands

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Leo J Hofland ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, Erasmus Medical Center, Rotterdam, The Netherlands

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Emanuel Christ ENETS Center of Excellence, Department of Endocrinology, University Hospital Basel, Basel, Switzerland
Department of Clinical Research, University of Basel, Basel, Switzerland

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Wouter W de Herder ENETS Center of Excellence, Department of Internal Medicine, Section of Endocrinology, 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, Rotterdam, The Netherlands

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Introduction Neuroendocrine neoplasms (NENs) consist of a diverse group of tumors arising from neuroendocrine cells and are mainly localized in the intestine, pancreas and lung ( Hofland et al. 2020 ). Classification of NENs is based on

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J Hadoux Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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D Malka Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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D Planchard Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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J Y Scoazec Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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C Caramella Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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J Guigay Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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V Boige Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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S Leboulleux Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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P Burtin Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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A Berdelou Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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Y Loriot Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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P Duvillard Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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C N Chougnet Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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D Déandréis Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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M Schlumberger Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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I Borget Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France
Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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M Ducreux Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France
Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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E Baudin Departments of Nuclear Medicine and Endocrine Tumors, Digestive Oncology, Medical Oncology (Thoracic Group), Pathology, Radiology, Centre Antoine Lacassagne, Department of Urologic Oncology, Department of Endocrinology, Department of Biostatistics and Epidemiology, Faculté de Médecine, Gustave Roussy, 114 Rue Edouard Vaillant, F-94800 Villejuif Cedex, France

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, temozolomide chemotherapy combined with capecitabine and/or bevacizumab was also proposed as an alternative for patients with neuroendocrine neoplasms and high proliferative index ( Welin et al . 2011 ). However, the applicability of this regimen in patients

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Georgios K Dimitriadis The Arden NET CoE, Warwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism (WISDEM), University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK

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Anna Angelousi Division of Pathophysiology, National and Kapodistrian University of Athens Medical School, Athens, Greece

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Martin O Weickert The Arden NET CoE, Warwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism (WISDEM), University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK

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Harpal S Randeva The Arden NET CoE, Warwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism (WISDEM), University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK

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Gregory Kaltsas The Arden NET CoE, Warwickshire Institute for the Study of Diabetes, Endocrinology and Metabolism (WISDEM), University Hospitals of Coventry and Warwickshire NHS Trust, Coventry, UK
Division of Pathophysiology, National and Kapodistrian University of Athens Medical School, Athens, Greece
Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK

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

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Sulentic P Xu JM Grossman AB 2017 Thymic neuroendocrine neoplasms: biological behaviour and therapy . Neuroendocrinology [in press]. ( doi:10.1159/000472255 ) Johnson BE Damodaran A Rushin J Gross A Le PT Chen HC

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S M Sadowski Endocrine Surgery, Surgical Oncology Program, National Cancer Institute, NIH, Bethesda, Maryland, USA

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C R C Pieterman Department of Surgical Oncology, Section of Surgical Endocrinology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA
Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands

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N D Perrier Department of Surgical Oncology, Section of Surgical Endocrinology, University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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F Triponez Thoracic and Endocrine Surgery and Faculty of Medicine, University Hospitals of Geneva, Geneva, Switzerland

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G D Valk Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands

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. ( https://doi.org/10.1210/jc.2014-3659 ) Grolmusz VK Kovesdi A Borks K Igaz P Patocs A 2018 Prognostic relevance of proliferation-related miRNAs in pancreatic neuroendocrine neoplasms . European Journal of Endocrinology 179 219 – 228

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Klaas Van Den Heede Department of Surgery, Skåne University Hospital, Lund, Sweden
Department of Clinical Sciences, Lund University, Lund, Sweden
Department of General and Endocrine Surgery, OLV Clinic Aalst, Aalst, Belgium

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Swathikan Chidambaram Department of Surgery and Cancer, Imperial College London, London, UK
Department of Endocrine Surgery, Hammersmith Hospital, London, UK

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Sam Van Slycke Department of General and Endocrine Surgery, OLV Clinic Aalst, Aalst, Belgium
Department of Head and Skin, University Hospital Ghent, Ghent, Belgium
Department of General Surgery, AZ Damiaan, Ostend, Belgium

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Nele Brusselaers Center for Translational Microbiome Research Department of Microbiology, Tumor and Cell Biology, Karolinska Institutet, Stockholm, Sweden
Global Health Institute, University of Antwerp, Wilrijk, Belgium

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Carl Fredrik Warfvinge Department of Clinical Sciences, Lund University, Lund, Sweden
Department of Oncology and Pathology, Lund University, Lund, Sweden

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Håkan Ohlsson Department of Clinical Sciences, Lund University, Lund, Sweden
Department of Surgery, Ystad Hospital, Ystad, Sweden

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Rita Gustafsson Department of Gastroenterology and Hepatology, Lund University, Lund, Sweden

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Erik Nordenström Department of Surgery, Skåne University Hospital, Lund, Sweden
Department of Clinical Sciences, Lund University, Lund, Sweden

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Martin Almquist Department of Surgery, Skåne University Hospital, Lund, Sweden
Department of Clinical Sciences, Lund University, Lund, Sweden

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patients with small bowel neuroendocrine neoplasms in the Netherlands . World Journal of Surgery 45 2482 – 2491 . ( https://doi.org/10.1007/s00268-021-06119-y ) Landerholm K Zar N Andersson RE Falkmer SE Jarhult J 2011 Survival and prognostic

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Atsuko Kasajima Department of Pathology, Technical University Munich, Munich, Germany
Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, Japan

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Yuichi Ishikawa Pathology Department, The Cancer Institute Hospital of JFCR, Tokyo, Japan

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Ayaka Iwata Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, Japan

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Katja Steiger Department of Pathology, Technical University Munich, Munich, Germany

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Naomi Oka Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, Japan
National Hospital Organization, Sendai Medical Center, Sendai, Japan

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Hirotaka Ishida Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, Japan

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Akira Sakurada Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University Graduate School of Medicine, Sendai, Japan

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Hiroyoshi Suzuki National Hospital Organization, Sendai Medical Center, Sendai, Japan

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Toru Kameya Division of Pathology, Shizuoka Cancer Center Hospital and Research Institute, Sizuoka, Japan

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Björn Konukiewitz Department of Pathology, Technical University Munich, Munich, Germany

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Günter Klöppel Department of Pathology, Technical University Munich, Munich, Germany

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Yoshinori Okada Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University Graduate School of Medicine, Sendai, Japan

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Hironobu Sasano Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, Japan

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Wilko Weichert Department of Pathology, Technical University Munich, Munich, Germany
Member of the German Cancer Consortium (DKTK)

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likely to metastasize and have a worse prognosis, these two neoplasms share the same phenotype and genotype ( Simbolo et al . 2017 ). In contrast to carcinoids, SCLC and LCNEC are high-grade neuroendocrine carcinomas (NEC) ( Travis et al . 2015

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Jaydira Del Rivero Developmental Therapeutics Branch, National Cancer Institute, National Institutes of Health, Bethesda, USA

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Josh Mailman NorCal CarciNET Community, Oakland, California, USA

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Michael W Rabow Department of Internal Medicine, Division of Palliative Medicine, University of California, San Francisco, San Francisco, California, USA

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Jennifer A Chan Harvard Medical School, Program in Carcinoid and Neuroendocrine Tumors, Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA

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Sarah Creed Good Shepherd Community Care, Harvard Kennedy School, Natick, Massachusetts, USA

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Hagen F Kennecke Providence Cancer Institute Franz Clinic, Portland Providence Medical Center, Portland, Oregon, USA

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Janice Pasieka Department of Surgery, Section of General Surgery, University of Calgary, Cumming School of Medicine, Calgary, Canada

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Jennifer Zuar Department of Internal Medicine, Division of Geriatrics and Palliative Medicine, Alpert Medical School, Providence, Rhode Island, USA

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Simron Singh Odette Cancer Centre, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada

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Lauren Fishbein Department of Medicine, Division of Endocrinology, Metabolism and Diabetes, Department of Biomedical Informatics, University of Colorado School of Medicine, Aurora, Colorado, USA

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relation to benefits to their patients. Summary Patients with neuroendocrine neoplasms have unique disease-related symptoms that impact palliative care needs during routine disease management and at the end of life. There are special considerations

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M Cives Department of Gastrointestinal Oncology, Department of Medicine, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Drive, Tampa, Florida 33612, USA

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P L Kunz Department of Gastrointestinal Oncology, Department of Medicine, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Drive, Tampa, Florida 33612, USA

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B Morse Department of Gastrointestinal Oncology, Department of Medicine, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Drive, Tampa, Florida 33612, USA

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D Coppola
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M J Schell Department of Gastrointestinal Oncology, Department of Medicine, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Drive, Tampa, Florida 33612, USA

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T Campos Department of Gastrointestinal Oncology, Department of Medicine, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Drive, Tampa, Florida 33612, USA

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P T Nguyen Department of Gastrointestinal Oncology, Department of Medicine, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Drive, Tampa, Florida 33612, USA

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P Nandoskar Department of Gastrointestinal Oncology, Department of Medicine, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Drive, Tampa, Florida 33612, USA

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V Khandelwal Department of Gastrointestinal Oncology, Department of Medicine, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Drive, Tampa, Florida 33612, USA

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J R Strosberg Department of Gastrointestinal Oncology, Department of Medicine, H. Lee Moffitt Cancer Center and Research Institute, 12902 Magnolia Drive, Tampa, Florida 33612, USA

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Introduction Neuroendocrine tumors (NETs) are a heterogeneous group of neoplasms characterized by a relatively indolent rate of growth and a propensity to produce and secrete a variety of hormones that give rise to diverse clinical syndromes

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