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Andreas Venizelos K.G. Jebsen Center for Genome-Directed Cancer Therapy, Department of Clinical Science, University of Bergen, Bergen, Norway
Department of Oncology, Haukeland University Hospital, Bergen, Norway

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Hege Elvebakken Department of Oncology, Ålesund Hospital, Møre og Romsdal Hospital Trust, Ålesund, Norway
Department of Clinical and Molecular Medicine, Faculty of Medicine and Health Sciences, Norwegian University of Science and Technology, Trondheim, Norway

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

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Oleksii Nikolaienko K.G. Jebsen Center for Genome-Directed Cancer Therapy, Department of Clinical Science, University of Bergen, Bergen, Norway
Department of Oncology, Haukeland University Hospital, Bergen, Norway

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Wei Deng K.G. Jebsen Center for Genome-Directed Cancer Therapy, Department of Clinical Science, University of Bergen, Bergen, Norway
Department of Oncology, Haukeland University Hospital, Bergen, Norway

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Inger Marie B Lothe Department of Pathology, Oslo University Hospital, Oslo, Norway

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Anne Couvelard Department of Pathology, Université de Paris, Bichat Hospital, AP-HP, Paris, France

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Geir Olav Hjortland Department of Oncology, Oslo University Hospital, Oslo, Norway

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Anna Sundlöv Departmentt of Oncology, Skåne University Hospital, Lund, Sweden
Department of Medical Radiation Physics, Lund University, Lund, Sweden

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Johanna Svensson Department of Oncology, Sahlgrenska University Hospital, Gothenburg, Sweden

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Harrish Garresori Department of Oncology, Stavanger University Hospital, Stavanger, Norway

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Christian Kersten Department of Research, Hospital of Southern Norway, Kristiansand, Norway

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Eva Hofsli Department of Clinical and Molecular Medicine, Faculty of Medicine and Health Sciences, Norwegian University of Science and Technology, Trondheim, Norway
Department of Oncology, St.Olavs Hospital, Trondheim, Norway

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Sönke Detlefsen Department of Pathology, Odense University Hospital, Odense, Denmark
Department of Clinical Medicine, Faculty of Health Sciences, University of Southern Denmark, Odense, Denmark

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Merete Krogh Department of Oncology, Odense University Hospital, Odense, Denmark

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Halfdan Sorbye Department of Oncology, Haukeland University Hospital, Bergen, Norway
Department of Clinical Science, University of Bergen, Bergen, Norway

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Stian Knappskog K.G. Jebsen Center for Genome-Directed Cancer Therapy, Department of Clinical Science, University of Bergen, Bergen, Norway
Department of Oncology, Haukeland University Hospital, Bergen, Norway

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Introduction High-grade (HG) gastroenteropancreatic (GEP) neuroendocrine neoplasms (NEN) are defined by the presence of neuroendocrine phenotype and a high proliferation rate (Ki-67 > 20%). The HG NEN entity consists of well

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Andreas Venizelos K.G. Jebsen Center for Genome-Directed Cancer Therapy, Department of Clinical Science, University of Bergen, Bergen, Norway
Department of Oncology, Haukeland University Hospital, Bergen, Norway

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Halfdan Sorbye Department of Oncology, Haukeland University Hospital, Bergen, Norway
Department of Clinical Science, University of Bergen, Bergen, Norway

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Hege Elvebakken Department of Oncology, Ålesund Hospital, Møre og Romsdal Hospital Trust, Ålesund, Norway
Department of Clinical and Molecular Medicine, Faculty of Medicine and Health Sciences, Norwegian University of Science and Technology, Trondheim, Norway

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Aurel Perren Institute of Tissue Medicine and Pathology, University of Bern, Bern, Switzerland

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Inger Marie B Lothe Department of Pathology, Oslo University Hospital, Oslo, Norway

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Anne Couvelard Department of Pathology, Université Paris Cité and AP-HP, Bichat Hospital, Paris, France

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Geir Olav Hjortland Department of Oncology, Oslo University Hospital, Oslo, Norway

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Anna Sundlöv Departmentt of Oncology, Skåne University Hospital, Lund, Sweden
Department of Medical Radiation Physics, Lund University, Lund, Sweden

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Johanna Svensson Department of Oncology, Sahlgrenska University Hospital, Gothenburg, Sweden

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Harrish Garresori Department of Oncology, Stavanger University Hospital, Stavanger, Norway

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Christian Kersten Department of Research, Hospital of Southern Norway, Kristiansand, Norway

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Eva Hofsli Department of Clinical and Molecular Medicine, Faculty of Medicine and Health Sciences, Norwegian University of Science and Technology, Trondheim, Norway
Department of Oncology, St. Olavs Hospital, Trondheim, Norway

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Sönke Detlefsen Department of Pathology, Odense University Hospital, Odense, Denmark
Department of Clinical Research, Faculty of Health Sciences, University of Southern Denmark, Odense, Denmark

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Lene W Vestermark Department of Oncology, Odense University Hospital, Odense, Denmark

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Morten Ladekarl Department of Oncology, Aarhus University Hospital, Aarhus, Denmark
Department of Oncology, Clinical Cancer Research Center, Aalborg University Hospital, Aalborg, Denmark

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Elizaveta Mitkina Tabaksblat Department of Oncology, Clinical Cancer Research Center, Aalborg University Hospital, Aalborg, Denmark

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Stian Knappskog K.G. Jebsen Center for Genome-Directed Cancer Therapy, Department of Clinical Science, University of Bergen, Bergen, Norway
Department of Oncology, Haukeland University Hospital, Bergen, Norway

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Introduction Neuroendocrine neoplasms (NENs) constitute ~2% of all malignancies and are frequently located in the gastrointestinal (GI) tract and pancreas. High-grade gastroenteropancreatic (HG-GEP) NENs are among the most aggressive cancers

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Jennifer R Eads Division of Hematology and Oncology, Abramson Cancer Center, University of Pennsylvania, Pennsylvania, USA

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Thorvardur R Halfdanarson Division of Medical Oncology, Mayo Clinic Cancer Center, Rochester, Minnesota, USA

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Tim Asmis Division of Medical Oncology, University of Ottawa, Ottawa, Ontario, Canada

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Andrew M Bellizzi Department of Pathology, University of Iowa Carver College of Medicine, Iowa City, Iowa, USA

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Emily K Bergsland Department of Medicine, University of California, San Francisco, California, USA

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Arvind Dasari Division of Gastrointestinal Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Ghassan El-Haddad Department of Diagnostic Imaging and Interventional Radiology, Moffitt Cancer Center and Research Institute, Tampa, Florida, USA

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Michael Frumovitz Division of Gynecologic Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Joshua Meyer Department of Radiation Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA

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Erik Mittra Division of Molecular Imaging and Therapy, Oregon Health & Science University, Portland, Oregon, USA

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Sten Myrehaug Department of Radiation Oncology, Odette Cancer Centre, Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada

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Eric Nakakura Department of Surgery, University of California, San Francisco, California, USA

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Nitya Raj Department of Medicine, Gastrointestinal Oncology Service, Memorial Sloan Kettering Cancer Center, New York, New York, USA

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Heloisa P Soares Division of Oncology, Huntsman Cancer Institute, University of Utah, Salt Lake City, Salt Lake City, Utah, USA

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Brian Untch Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York, USA

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Namrata Vijayvergia Department of Hematology and Oncology, Fox Chase Cancer Center, Philadelphia, Pennsylvania, USA

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Jennifer A Chan Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA

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management of high-grade NENs of gastroenteropancreatic and gynecologic origin. Methods for developing consensus practice recommendations A panel consisting of 17 neuroendocrine experts from 13 institutions was convened by the NANETS Publications and

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Tobias Hofving Department of Laboratory Medicine, Institute of Biomedicine, Sahlgrenska Cancer Center, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden

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Viktor Sandblom Department of Radiation Physics, Institute of Clinical Sciences, Sahlgrenska Cancer Center, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden

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Yvonne Arvidsson Department of Laboratory Medicine, Institute of Biomedicine, Sahlgrenska Cancer Center, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden

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Emman Shubbar Department of Radiation Physics, Institute of Clinical Sciences, Sahlgrenska Cancer Center, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden

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Gülay Altiparmak Department of Laboratory Medicine, Institute of Biomedicine, Sahlgrenska Cancer Center, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden

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John Swanpalmer Department of Radiation Physics, Institute of Clinical Sciences, Sahlgrenska Cancer Center, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden
Department of Medical Physics and Biomedical Engineering, Sahlgrenska University Hospital, Gothenburg, Sweden

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Bilal Almobarak Department of Laboratory Medicine, Institute of Biomedicine, Sahlgrenska Cancer Center, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden

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Anna-Karin Elf Department of Surgery, Institute of Clinical Sciences, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden

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Viktor Johanson Department of Surgery, Institute of Clinical Sciences, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden

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Erik Elias Department of Surgery, Institute of Clinical Sciences, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden

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Erik Kristiansson Department of Mathematical Sciences, Chalmers University of Technology, Gothenburg, Sweden

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Eva Forssell-Aronsson Department of Radiation Physics, Institute of Clinical Sciences, Sahlgrenska Cancer Center, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden
Department of Medical Physics and Biomedical Engineering, Sahlgrenska University Hospital, Gothenburg, Sweden

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Ola Nilsson Department of Laboratory Medicine, Institute of Biomedicine, Sahlgrenska Cancer Center, Sahlgrenska Academy at University of Gothenburg, Gothenburg, Sweden

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months) in patients with small intestinal neuroendocrine tumours (SINETs), compared with the best standard of care ( Strosberg et al. 2017 ). This has led to an FDA approval of 177 Lu-octreotate therapy for gastroenteropancreatic NETs and its inclusion

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Tobias Hofving Sahlgrenska Cancer Center, Department of Pathology and Genetics, Institute of Biomedicine, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden

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Yvonne Arvidsson Sahlgrenska Cancer Center, Department of Pathology and Genetics, Institute of Biomedicine, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden

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Bilal Almobarak Sahlgrenska Cancer Center, Department of Pathology and Genetics, Institute of Biomedicine, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden

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Linda Inge Sahlgrenska Cancer Center, Department of Pathology and Genetics, Institute of Biomedicine, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden

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Roswitha Pfragner Institute of Pathophysiology and Immunology, Center for Molecular Medicine, Medical University of Graz, Graz, Austria

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Marta Persson Sahlgrenska Cancer Center, Department of Pathology and Genetics, Institute of Biomedicine, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden

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Göran Stenman Sahlgrenska Cancer Center, Department of Pathology and Genetics, Institute of Biomedicine, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden

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Erik Kristiansson Department of Mathematical Sciences, Chalmers University of Technology, Gothenburg, Sweden

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Viktor Johanson Department of Surgery, Institute of Clinical Sciences, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden

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Ola Nilsson Sahlgrenska Cancer Center, Department of Pathology and Genetics, Institute of Biomedicine, Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden

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gastroenteropancreatic neuroendocrine tumors. Journal of the National Cancer Institute 100 1282 – 1289 . ( https://doi.org/10.1093/jnci/djn275 ) 18780869 10.1093/jnci/djn275 Perren A Komminoth P Saremaslani P Matter C Feurer S Lees JA Heitz PU Eng C 2000

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Dimitrios Papantoniou Department of Medical Sciences, Endocrine Oncology, Uppsala University, Uppsala, Sweden
Department of Oncology, Ryhov County Hospital, Jönköping, Sweden

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Malin Grönberg Department of Medical Sciences, Endocrine Oncology, Uppsala University, Uppsala, Sweden

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Espen Thiis-Evensen Oslo University Hospital, Rikshospitalet, Deptartment of Organ Transplant, Oslo, Norway

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Halfdan Sorbye Haukeland Hospital, Deptartment of Oncology, Bergen, Norway
University of Bergen, Deptartment of Clinical Medicine, Bergen, Norway

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Kalle Landerholm Department of Biomedical and Clinical Sciences, Linköping University, Linköping, Sweden
Department of Surgery, Ryhov County Hospital, Jönköping, Sweden

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Staffan Welin Department of Medical Sciences, Endocrine Oncology, Uppsala University, Uppsala, Sweden

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Eva Tiensuu Janson Department of Medical Sciences, Endocrine Oncology, Uppsala University, Uppsala, Sweden

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C Schade-Brittinger C Barth P Moll R 2005 Octreotide versus octreotide plus interferon-alpha in endocrine gastroenteropancreatic tumors: a randomized trial . Clinical Gastroenterology and Hepatology 3 761 – 771 . ( https://doi.org/10.1016/S

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Anna Koumarianou Medical Oncology Unit, Endocrine Unit, MRI Unit, Pathology Department, Fourth Department of Surgery, Medical Oncology Unit, Second Department of Internal Medicine Propaedeutic, Attikon University General Hospital, Rimini 1, 12462 Athens, Greece

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Stavroula Antoniou Medical Oncology Unit, Endocrine Unit, MRI Unit, Pathology Department, Fourth Department of Surgery, Medical Oncology Unit, Second Department of Internal Medicine Propaedeutic, Attikon University General Hospital, Rimini 1, 12462 Athens, Greece

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George Kanakis Medical Oncology Unit, Endocrine Unit, MRI Unit, Pathology Department, Fourth Department of Surgery, Medical Oncology Unit, Second Department of Internal Medicine Propaedeutic, Attikon University General Hospital, Rimini 1, 12462 Athens, Greece

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Nikolaos Economopoulos Medical Oncology Unit, Endocrine Unit, MRI Unit, Pathology Department, Fourth Department of Surgery, Medical Oncology Unit, Second Department of Internal Medicine Propaedeutic, Attikon University General Hospital, Rimini 1, 12462 Athens, Greece

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Dimitra Rontogianni Medical Oncology Unit, Endocrine Unit, MRI Unit, Pathology Department, Fourth Department of Surgery, Medical Oncology Unit, Second Department of Internal Medicine Propaedeutic, Attikon University General Hospital, Rimini 1, 12462 Athens, Greece

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Anastasios Ntavatzikos Medical Oncology Unit, Endocrine Unit, MRI Unit, Pathology Department, Fourth Department of Surgery, Medical Oncology Unit, Second Department of Internal Medicine Propaedeutic, Attikon University General Hospital, Rimini 1, 12462 Athens, Greece

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Nikolaos Tsavaris Medical Oncology Unit, Endocrine Unit, MRI Unit, Pathology Department, Fourth Department of Surgery, Medical Oncology Unit, Second Department of Internal Medicine Propaedeutic, Attikon University General Hospital, Rimini 1, 12462 Athens, Greece

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Dimitrios Pectasides Medical Oncology Unit, Endocrine Unit, MRI Unit, Pathology Department, Fourth Department of Surgery, Medical Oncology Unit, Second Department of Internal Medicine Propaedeutic, Attikon University General Hospital, Rimini 1, 12462 Athens, Greece

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George Dimitriadis Medical Oncology Unit, Endocrine Unit, MRI Unit, Pathology Department, Fourth Department of Surgery, Medical Oncology Unit, Second Department of Internal Medicine Propaedeutic, Attikon University General Hospital, Rimini 1, 12462 Athens, Greece

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Gregory Kaltsas Medical Oncology Unit, Endocrine Unit, MRI Unit, Pathology Department, Fourth Department of Surgery, Medical Oncology Unit, Second Department of Internal Medicine Propaedeutic, Attikon University General Hospital, Rimini 1, 12462 Athens, Greece

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Downstaging of gastroenteropancreatic tumours (arrows) after TeBeSa combination therapy. MRI details shown before treatment (a) and (c) and after six cycles (b) and (d) for two patients with a pancreatic (a)/(b) and a duodenal tumour (c)/(d). During the past 4

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Paula Fontes Asprino Hospital Sírio-Libanês (HSL), São Paulo, São Paulo, Brazil

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Rudinei Diogo Marques Linck Hospital Sírio-Libanês (HSL), São Paulo, São Paulo, Brazil
Instituto do Câncer do Estado de São Paulo (ICESP), São Paulo, São Paulo, Brazil

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Jônatas Cesar Instituto de Biociências – Universidade de São Paulo (IB-USP), São Paulo, São Paulo, Brazil

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Florêncio Porto Freitas Hospital Sírio-Libanês (HSL), São Paulo, São Paulo, Brazil

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Fernanda Christtanini Koyama Hospital Sírio-Libanês (HSL), São Paulo, São Paulo, Brazil

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Rachel Simões Pimenta Riechelmann Instituto do Câncer do Estado de São Paulo (ICESP), São Paulo, São Paulo, Brazil

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Frederico Perego Costa Hospital Sírio-Libanês (HSL), São Paulo, São Paulo, Brazil

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Paulo Marcelo Gehm Hoff Hospital Sírio-Libanês (HSL), São Paulo, São Paulo, Brazil
Instituto do Câncer do Estado de São Paulo (ICESP), São Paulo, São Paulo, Brazil

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Pedro Alexandre Favoretto Galante Hospital Sírio-Libanês (HSL), São Paulo, São Paulo, Brazil

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Diogo Meyer Instituto de Biociências – Universidade de São Paulo (IB-USP), São Paulo, São Paulo, Brazil

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Anamaria Aranha Camargo Hospital Sírio-Libanês (HSL), São Paulo, São Paulo, Brazil

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Jorge Sabbaga Hospital Sírio-Libanês (HSL), São Paulo, São Paulo, Brazil
Instituto do Câncer do Estado de São Paulo (ICESP), São Paulo, São Paulo, Brazil

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, epidemiological studies have demonstrated that non-syndromic individuals with an affected relative are at an increased risk of developing gastroenteropancreatic (GEP) NENs. In individuals with affected siblings, the relative risk of GEP NEN increases by 13.4-fold

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Sara Pusceddu Department of Medical Oncology ENETS Center of Excellence, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Milan, Italy

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Francesco Barretta Unit of Clinical Epidemiology and Trial Organization, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, ENETS Center of Excellence, Milan, Italy

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Annalisa Trama Department of Preventive and Predictive Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Evaluative Epidemiology Unit, ENETS Center of Excellence, Milan, Italy

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Laura Botta Department of Preventive and Predictive Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Evaluative Epidemiology Unit, ENETS Center of Excellence, Milan, Italy

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Massimo Milione Department of Pathology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, ENETS Center of Excellence, Milan, Italy

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Roberto Buzzoni Department of Medical Oncology ENETS Center of Excellence, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Milan, Italy

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Filippo De Braud Department of Medical Oncology ENETS Center of Excellence, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, Milan, Italy
University of Milan, Milan, Italy

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Vincenzo Mazzaferro University of Milan, Milan, Italy
Liver Surgery, Transplantation and Gastroenterology, University of Milan and Istituto Nazionale Tumori Fondazione IRCCS, ENETS Center of Excellence, Milano, Milan, Italy

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Ugo Pastorino Department of Thoracic Surgical Oncology, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, ENETS Center of Excellence, Milan, Italy

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Ettore Seregni Department of Nuclear Medicine ENETS Center of Excellence, Fondazione IRCCS Istituto Nazionale dei Tumori, Milano, Milan, Italy

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Luigi Mariani Unit of Clinical Epidemiology and Trial Organization, Fondazione IRCCS Istituto Nazionale dei Tumori di Milano, ENETS Center of Excellence, Milan, Italy

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Jorgelina Coppa Liver Surgery, Transplantation and Gastroenterology, University of Milan and Istituto Nazionale Tumori Fondazione IRCCS, ENETS Center of Excellence, Milano, Milan, Italy

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Harry Comber Ireland National Cancer Registry, Cork, Ireland

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Antongiulio Faggiano Department of Thyroid and Parathyroid Surgery Unit, Azienda Ospedaliera Universitaria Federico II, ENETS Center of Excellence, Naples, Italy

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Kris Henau Belgian Cancer Registry, Brussels, Belgium

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Toni Ibrahim Osteoncology and Rare Tumors Center, Istituto Scientifico Romagnolo per lo Studio e la Cura dei Tumori IRST, IRCCS, Meldola, Italy

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Maja Primic Žakelj Institute of Oncology Ljubljana, Epidemiology and Cancer Registry, Ljubljana, Slovenia

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NEPscore Working Group
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Tumor Society (ENETS) Center of Excellence for the treatment of gastroenteropancreatic (GEP)-NETs; (ii) a European external validation cohort, which comprised rare NET patients included in the database of the pilot study of the European project

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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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, ease of administration, and tolerability, SSAs are generally considered as first-line therapeutic option in well-differentiated gastroenteropancreatic NETs (GEP-NETs). The role of continuing SSAs after disease progression is debatable ( Strosberg et al

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