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J Cros Department of Pathology, AP-HP, DHU UNITY, Beaujon University Hospital, Clichy, France
U1149 – University Paris Diderot, Paris, France

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O Hentic Department of Gastroenterology and Pancreatology, AP-HP, DHU UNITY, Beaujon University Hospital, Clichy, France

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V Rebours U1149 – University Paris Diderot, Paris, France
Department of Gastroenterology and Pancreatology, AP-HP, DHU UNITY, Beaujon University Hospital, Clichy, France

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M Zappa Department of Radiology, AP-HP, DHU UNITY, Beaujon Hospital, Clichy, France

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N Gille Department of Pathology, AP-HP, DHU UNITY, Beaujon University Hospital, Clichy, France

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N Theou-Anton Department of Somatic Genetic, AP-HP, DHU UNITY, Bichat University Hospital, Paris, France

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D Vernerey Methodology and Quality of Life in Oncology Unit (EA 3181), University Hospital of Besançon, Besançon, France

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F Maire Department of Gastroenterology and Pancreatology, AP-HP, DHU UNITY, Beaujon University Hospital, Clichy, France

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P Lévy U1149 – University Paris Diderot, Paris, France
Department of Gastroenterology and Pancreatology, AP-HP, DHU UNITY, Beaujon University Hospital, Clichy, France

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P Bedossa Department of Pathology, AP-HP, DHU UNITY, Beaujon University Hospital, Clichy, France
U1149 – University Paris Diderot, Paris, France

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V Paradis Department of Pathology, AP-HP, DHU UNITY, Beaujon University Hospital, Clichy, France
U1149 – University Paris Diderot, Paris, France

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P Hammel U1149 – University Paris Diderot, Paris, France
Department of Digestive Oncology, AP-HP, DHU UNITY, Beaujon University Hospital, Clichy, France

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P Ruszniewski U1149 – University Paris Diderot, Paris, France
Department of Gastroenterology and Pancreatology, AP-HP, DHU UNITY, Beaujon University Hospital, Clichy, France

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A Couvelard U1149 – University Paris Diderot, Paris, France
Department of Pathology, AP-HP, DHU UNITY, Bichat University Hospital, Paris, France

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Introduction Evidences suggesting the efficacy of temozolomide (TEM), a DNA-alkylating agent, in advanced pancreatic neuroendocrine tumors (PNETs) are accumulating ( Kulke et al. 2006 , Ekeblad et al. 2007 , Strosberg et al. 2011

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Simona Falletta Department of Medical Science, Section of Endocrinology and Internal Medicine, University of Ferrara, Ferrara, Italy

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Stefano Partelli Pancreatic Surgery Unit, Pancreas Translational and Research Institute, San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy

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Corrado Rubini Department of Biomedical Sciences and Public Health, Polytechnic University of Marche, Ancona, Italy

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Dominik Nann Institut fur Pathologie, University of Bern, Bern, Switzerland

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Andrea Doria Department of Biomedical Sciences and Public Health, Polytechnic University of Marche, Ancona, Italy

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Ilaria Marinoni Institut fur Pathologie, University of Bern, Bern, Switzerland

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Vanessa Polenta Pancreatic Surgery Unit, Pancreas Translational and Research Institute, San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy

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Carmelina Di Pasquale Department of Medical Science, Section of Endocrinology and Internal Medicine, University of Ferrara, Ferrara, Italy

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Ettore degli Uberti Department of Medical Science, Section of Endocrinology and Internal Medicine, University of Ferrara, Ferrara, Italy

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Aurel Perren Institut fur Pathologie, University of Bern, Bern, Switzerland

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Massimo Falconi Pancreatic Surgery Unit, Pancreas Translational and Research Institute, San Raffaele Hospital, Vita-Salute San Raffaele University, Milan, Italy

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

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Introduction Pancreatic neuroendocrine tumors (P-NETs) are heterogeneous neoplasms originating from pancreatic neuroendocrine cells ( Singh et al. 2015 , Berardi et al . 2016 ), representing 1–4% of all pancreatic malignancies, with an

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Brendan M Finnerty Department of Surgery, Weill Cornell Medicine, New York, New York, USA

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Maureen D Moore Department of Surgery, Weill Cornell Medicine, New York, New York, USA

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Akanksha Verma Department of Physiology and Biophysics, Weill Cornell Medicine, New York, New York, USA

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Anna Aronova Department of Surgery, Weill Cornell Medicine, New York, New York, USA

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Shixia Huang Dan L. Duncan Cancer Center and Department of Molecular and Cellular Biology, Baylor College of Medicine, One Baylor Plaza, Houston, Texas, USA

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Dean P Edwards Dan L. Duncan Cancer Center and Department of Molecular and Cellular Biology, Baylor College of Medicine, One Baylor Plaza, Houston, Texas, USA

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Zhengming Chen Department of Healthcare Policy & Research, Weill Cornell Medicine, New York, New York, USA

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Marco Seandel Department of Surgery, Weill Cornell Medicine, New York, New York, USA

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Theresa Scognamiglio Department of Pathology & Laboratory Medicine, Weill Cornell Medicine, New York, New York, USA

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Yi-Chieh Nancy Du Department of Pathology & Laboratory Medicine, Weill Cornell Medicine, New York, New York, USA

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Olivier Elemento Department of Physiology and Biophysics, Weill Cornell Medicine, New York, New York, USA

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Rasa Zarnegar Department of Surgery, Weill Cornell Medicine, New York, New York, USA

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Irene M Min Department of Surgery, Weill Cornell Medicine, New York, New York, USA

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Thomas J Fahey III Department of Surgery, Weill Cornell Medicine, New York, New York, USA

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Introduction Pancreatic neuroendocrine tumors (PNETs) are rare tumors comprising only 2% of all pancreatic tumors, with a twofold increase in incidence since 1975 ( Halfdanarson et al. 2008 , Lawrence et al. 2011 ). The role of genetic

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Mojun Zhu Department of Oncology, Mayo Clinic, Rochester, Minnesota, USA

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Karl R Sorenson Department of Oncology, Mayo Clinic, Rochester, Minnesota, USA

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Rebecca Liu Department of Medicine, Massachusetts General Hospital, Boston, Massachusetts, USA

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Bonnie E Gould Rothberg Smilow Cancer Hospital, Yale-New Haven Health System, New Haven, Connecticut, USA

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Thorvardur R Halfdanarson Department of Oncology, Mayo Clinic, Rochester, Minnesota, USA

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Introduction Pancreatic neuroendocrine tumors (PNETs) are a group of heterogeneous malignancies with diverse pathological findings and clinical behaviors. With an annual incidence that is less than 1 per 100,000 ( Halfdanarson et al. 2008

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Ophélie De Rycke Department of Gastroenterology and Pancreatology, ENETS Centre of Excellence, Beaujon University Hospital, Clichy, France
Université de Paris, Centre de Recherche sur l’Inflammation, INSERM, Paris, France

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Thomas Walter Department of Gastro-enterology and oncology, Hospices Civils de Lyon, Edouard Herriot University Hospital, Lyon, France

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Marine Perrier Department of Hepato-Gastroenterology and Digestive Oncology, Robert Debré University Hospital, Reims, France

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Olivia Hentic Department of Gastroenterology and Pancreatology, ENETS Centre of Excellence, Beaujon University Hospital, Clichy, France

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Catherine Lombard-Bohas Department of Gastro-enterology and oncology, Hospices Civils de Lyon, Edouard Herriot University Hospital, Lyon, France

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Romain Coriat Department of Gastroenterology, Cochin University Hospital, Paris, France

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Guillaume Cadiot Department of Hepato-Gastroenterology and Digestive Oncology, Robert Debré University Hospital, Reims, France

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Anne Couvelard Université de Paris, Centre de Recherche sur l’Inflammation, INSERM, Paris, France
Department of Pathology, ENETS Centre of Excellence, Bichat/Beaujon University Hospital, Paris/Clichy, France

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Philippe Ruszniewski Department of Gastroenterology and Pancreatology, ENETS Centre of Excellence, Beaujon University Hospital, Clichy, France
Université de Paris, Centre de Recherche sur l’Inflammation, INSERM, Paris, France

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Jérôme Cros Université de Paris, Centre de Recherche sur l’Inflammation, INSERM, Paris, France
Department of Pathology, ENETS Centre of Excellence, Bichat/Beaujon University Hospital, Paris/Clichy, France

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Louis de Mestier Department of Gastroenterology and Pancreatology, ENETS Centre of Excellence, Beaujon University Hospital, Clichy, France
Université de Paris, Centre de Recherche sur l’Inflammation, INSERM, Paris, France

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Introduction Pancreatic neuroendocrine tumors (PanNET) are a heterogeneous group of rare neoplasms. However, in the past few decades, their incidence has increased for all grades and stages ( Dasari et al. 2017 ). Approximately 20% of PanNET

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Sonia M Abuzakhm OhioHealth, Columbus, Ohio, USA

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Vineeth Sukrithan The Ohio State University Comprehensive Cancer Center, Columbus, Ohio, USA

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Briant Fruth Mayo Clinic, Rochester, Minnesota, USA

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Rui Qin Janssen Pharmaceuticals, Raritan, New Jersey, USA

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Jonathan Strosberg H. Lee Moffitt Cancer Center & Research Institute, Tampa, Florida, USA

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Timothy J Hobday Mayo Clinic, Rochester, Minnesota, USA

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Thomas Semrad Tahoe Forest Cancer Center, Truckee, California, USA

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Diane Reidy-Lagunes Memorial Sloan Kettering Cancer Center, New York, New York, USA

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Hedy Lee Kindler University of Chicago Medical Center, Chicago, Illinois, USA

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George P Kim George Washington University Cancer Center, Washington, DC, USA

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Jennifer J Knox Princess Margaret Cancer Centre, Toronto, ON, Canada

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Andreas Kaubisch Montefiore Medical Center, Bronx, New York, USA

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Miguel Villalona-Calero City of Hope, Duarte, California, USA

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Helen Chen CTEP National Cancer Institute, Bethesda, Maryland, USA

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Charles Erlichman Mayo Clinic, Rochester, Minnesota, USA

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Manisha H Shah The Ohio State University Comprehensive Cancer Center, Columbus, Ohio, USA

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patients with advanced extra-pancreatic neuroendocrine tumors. Around 54% of patients discontinued treatment due to adverse events, refusal of further treatment, or treatment delays. Given the increased toxicity observed with the combination, particularly

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Olga Lakiza Endocrine and Neuroendocrine Surgery Research Program, Division of General Surgery and Surgical Oncology, Department of Surgery, University of Chicago Medicine, Chicago, Illinois, USA

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Julian Lutze Committee on Cancer Biology, University of Chicago, Chicago, Illinois, USA

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Alyx Vogle Endocrine and Neuroendocrine Surgery Research Program, Division of General Surgery and Surgical Oncology, Department of Surgery, University of Chicago Medicine, Chicago, Illinois, USA

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Jelani Williams Endocrine and Neuroendocrine Surgery Research Program, Division of General Surgery and Surgical Oncology, Department of Surgery, University of Chicago Medicine, Chicago, Illinois, USA

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Abde Abukdheir Division of Hematology, Oncology, and Cell Therapy, Department of Internal Medicine, Rush University Medical Center, Chicago, Illinois, USA

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Paul Miller Endocrine and Neuroendocrine Surgery Research Program, Division of General Surgery and Surgical Oncology, Department of Surgery, University of Chicago Medicine, Chicago, Illinois, USA

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Chih-Yi ‘Andy’ Liao Division of Hematology and Oncology, Department of Internal Medicine, University of Chicago, Chicago, Illinois, USA

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Sean P Pitroda Department of Radiation Oncology and Cellular Biology, University of Chicago, Chicago, Illinois, USA

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Carlos Martinez Department of Radiation Oncology and Cellular Biology, University of Chicago, Chicago, Illinois, USA

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Andrea Olivas Department of Pathology, University of Chicago, Chicago, Illinois, USA

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Namrata Setia Department of Pathology, University of Chicago, Chicago, Illinois, USA

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Stephen J Kron Committee on Cancer Biology, University of Chicago, Chicago, Illinois, USA
Department of Molecular Genetics and Cell Biology, University of Chicago, Chicago, Illinois, USA

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Ralph R Weichselbaum Department of Radiation Oncology and Cellular Biology, University of Chicago, Chicago, Illinois, USA
Ludwig Center for Metastasis Research, University of Chicago, Chicago, Illinois, USA

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Xavier M Keutgen Endocrine and Neuroendocrine Surgery Research Program, Division of General Surgery and Surgical Oncology, Department of Surgery, University of Chicago Medicine, Chicago, Illinois, USA

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Introduction Pancreatic neuroendocrine tumors (PanNET) are rare, but their incidence is steadily increasing. Patients with PanNETs present with metastatic disease in 50–70% of cases and less than 50% will survive longer than 5 years

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M Cives Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center and Research Institute

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M Ghayouri Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center and Research Institute

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B Morse Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center and Research Institute

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M Brelsford Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center and Research Institute

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M Black Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center and Research Institute

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A Rizzo Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

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A Meeker Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

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J Strosberg Department of Gastrointestinal Oncology, H. Lee Moffitt Cancer Center and Research Institute

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Introduction Chemotherapy regimens containing the oral alkylating agent temozolomide are active in the treatment of metastatic pancreatic neuroendocrine tumors (pNETs), with response rates ranging from 30 to 70% ( Kulke et al . 2006

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Margaux Foulfoin Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de Gastroentérologie et d’Oncologie Médicale, Lyon, France

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Emmanuelle Graillot Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de Gastroentérologie et d’Oncologie Médicale, Lyon, France
University of Lyon, Université Lyon 1, Lyon, France

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Mustapha Adham University of Lyon, Université Lyon 1, Lyon, France
Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de chirurgie, Lyon, France

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Pascal Rousset University of Lyon, Université Lyon 1, Lyon, France
Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de radiologie, Lyon, France

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Julien Forestier Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de Gastroentérologie et d’Oncologie Médicale, Lyon, France

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Valérie Hervieu University of Lyon, Université Lyon 1, Lyon, France
Hospices Civils de Lyon, Hôpital Edouard Herriot, Service Central d’Anatomie et Cytologie Pathologiques, Lyon, France

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Philip Robinson Hospices Civils de Lyon, DRCI, Lyon, France

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Jean-Yves Scoazec Hospices Civils de Lyon, Hôpital Edouard Herriot, Service Central d’Anatomie et Cytologie Pathologiques, Lyon, France

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Catherine Lombard-Bohas Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de Gastroentérologie et d’Oncologie Médicale, Lyon, France

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Thomas Walter Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de Gastroentérologie et d’Oncologie Médicale, Lyon, France
University of Lyon, Université Lyon 1, Lyon, France

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Introduction The prevalence of pancreatic neuroendocrine tumors (P-NET) is 0.32 per 100,000 in the US, and the incidence is rising ( Yao et al . 2008 ). The median survival is longer than that in pancreatic adenocarcinoma, and patients

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Tobias Krauss Department of Radiology, Medical Center – University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany

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Alfonso Massimiliano Ferrara Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology IOV- IRCCS, Padua, Italy

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Thera P Links Department of Endocrinology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands

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Ulrich Wellner Department of Surgery, University of Luebeck, Luebeck, Germany

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Irina Bancos Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, USA

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Andrey Kvachenyuk Institute of Endocrinology and Metabolism, NAMS of Ukraine, Kiev, Ukraine

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Karina Villar Gómez de las Heras Central Services, Servicio de Salud de Castilla-La Mancha (SESCAM), Toledo, Spain

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Marina Y Yukina Department of Surgery, Endocrinology Research Center, Moscow, Russia

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Roman Petrov Department of Surgery, Bakhrushin Brothers Moscow City Hospital, Moscow, Russia

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Garrett Bullivant Princess Margaret Cancer Center, University Health Network, Toronto, Ontario, Canada

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Laura von Duecker Department of Medicine IV, Faculty of Medicine, Albert-Ludwigs-University, Freiburg, Germany

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Swati Jadhav Department of Endocrinology, KEM Hospital, Mumbai, India

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Ursula Ploeckinger Interdisciplinary Center of Metabolism: Endocrinology, Diabetes and Metabolism, Charité-University Medicine Berlin, Campus Virchow-Klinikum, Berlin, Germany

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Staffan Welin Department of Endocrine Oncology, Uppsala University Hospital, Uppsala, Sweden

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

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Oliver Gimm Department of Clinical and Experimental Medicine, Department of Surgery, University of Linköping, Linköping, Sweden

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Marija Pfeifer Department of Endocrinology, University Medical Center, Ljubljana, Slovenia

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Joanne Ngeow Cancer Genetics Service, Division of Medical Oncology, National Cancer Center Singapore and Lee Kong Chian School of Medicine, Nanyang Technological University, Singapore, Singapore

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Kornelia Hasse-Lazar Department of Endocrine Oncology and Nuclear Medicine, Center of Oncology, MSC Memorial Institute, Gliwice, Poland

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Gabriela Sansó Centro de Investigaciones Endocrinológicas “Dr Cesar Bergada” (CEDIE), Hospital de Niños Ricardo Gutiérrez, CABA, Buenos Aires, Argentina

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Xiaoping Qi Department of Oncologic and Urologic Surgery, the 117th PLA Hospital, Wenzhou Medical University, Hangzhou, Peoples Republic of China

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M Umit Ugurlu Department of General Surgery, Breast and Endocrine Surgery Unit, Marmara University School of Medicine, Istanbul, Turkey

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Rene E Diaz Endocrine Section, Hospital del Salvador, Santiago de Chile, Chile

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Nelson Wohllk Department of Medicine, Endocrine Section, Hospital del Salvador, University of Chile, Santiago de Chile, Chile

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Mariola Peczkowska Department of Hypertension, Institute of Cardiology, Warsaw, Poland

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Jens Aberle 3rd Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany

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Maria A A Pereira Serviço de Endocrinologia, Hospital das Clinicas (HCFMUSP), Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

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Ana O Hoff Serviço de Endocrinologia, Hospital das Clínicas (HCFMUSP) and Instituto do Cancer do Estado de São Paulo (ICESP), Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

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Alice H D Violante Department of Internal Medicine-Endocrinology, Faculty of medicine-Hospital Universitario Clementino Fraga Filho, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil

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Zhewei Zhang Department of Urology, 2nd Hospital of Zhejiang University, School of Medicine, Hangzhou, China

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Tada Kunavisarut Division of Endocrinology and metabolism, Siriraj Hospital, Mahidol University, Bangkok, Thailand

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Simona Grozinsky-Glasberg Neuroendocrine Tumor Division, Endocrinology & Metabolism Service, Department of Medicine, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

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Merav Fraenkel Neuroendocrine Tumor Division, Endocrinology & Metabolism Service, Department of Medicine, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

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Matthias Schott Department of Endocrinology, Heinrich-Heine-University, Düsseldorf, Germany

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Juri Ruf Department of Nuclear Medicine, Faculty of Medicine, Albert-Ludwigs-University, Freiburg, Germany

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Stefania Zovato Familial Cancer Clinic and Oncoendocrinology, Veneto Institute of Oncology IOV- IRCCS, Padua, Italy

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Ernst von Dobschuetz Section of Endocrine Surgery, Reinbek Hospital, Academic Teaching Hospital University of Hamburg, Reinbek, Germany

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Holger Amthauer Department of Clinical Nuclear Medicine, Charité – Universitätsmedizin Berlin, Berlin, Germany

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Özer Makay Department of General Surgery, Division of Endocrine Surgery, Izmir, Turkey

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William F Young Jr Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, USA

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). Pancreatic neuroendocrine tumors (PanNETs) originate from the islets of the pancreas and may be hormonally active or inactive. PanNETs occur mainly as sporadic tumors, but 9% are also components of three hereditary syndromes, multiple endocrine neoplasia type

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