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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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exhibit a very heterogeneous prognosis. Pathological differentiation or grading and TNM stage are the most important prognostic factors ( Baudin 2007 ). Poorly differentiated neuroendocrine carcinomas (NECs) are characterized by a poorer outcome when

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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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Introduction Pulmonary neuroendocrine tumors, as defined in the 2015 classification of the World Health Organization (WHO), comprise three different types; carcinoid tumors, large cell neuroendocrine carcinomas (LCNEC) and small-cell lung

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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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-differentiated high-grade neuroendocrine tumors (G3 NET) and poorly differentiated high-grade neuroendocrine carcinomas (NECs). Most available literature does not account for this subcategorization and as a result, much of the management of high-grade NENs is based on

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Nora Sahnane Section of Anatomic Pathology, CRIBI Biotechnology Center, Department of Molecular Medicine, Department of Pathology, Department of Surgical and Morphological Sciences, University of Insubria, Via O. Rossi, 9, 21100 Varese, Italy

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Daniela Furlan Section of Anatomic Pathology, CRIBI Biotechnology Center, Department of Molecular Medicine, Department of Pathology, Department of Surgical and Morphological Sciences, University of Insubria, Via O. Rossi, 9, 21100 Varese, Italy

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Matilde Monti Section of Anatomic Pathology, CRIBI Biotechnology Center, Department of Molecular Medicine, Department of Pathology, Department of Surgical and Morphological Sciences, University of Insubria, Via O. Rossi, 9, 21100 Varese, Italy

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Chiara Romualdi Section of Anatomic Pathology, CRIBI Biotechnology Center, Department of Molecular Medicine, Department of Pathology, Department of Surgical and Morphological Sciences, University of Insubria, Via O. Rossi, 9, 21100 Varese, Italy

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Alessandro Vanoli Section of Anatomic Pathology, CRIBI Biotechnology Center, Department of Molecular Medicine, Department of Pathology, Department of Surgical and Morphological Sciences, University of Insubria, Via O. Rossi, 9, 21100 Varese, Italy

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Emanuela Vicari Section of Anatomic Pathology, CRIBI Biotechnology Center, Department of Molecular Medicine, Department of Pathology, Department of Surgical and Morphological Sciences, University of Insubria, Via O. Rossi, 9, 21100 Varese, Italy

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Enrico Solcia Section of Anatomic Pathology, CRIBI Biotechnology Center, Department of Molecular Medicine, Department of Pathology, Department of Surgical and Morphological Sciences, University of Insubria, Via O. Rossi, 9, 21100 Varese, Italy

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Carlo Capella Section of Anatomic Pathology, CRIBI Biotechnology Center, Department of Molecular Medicine, Department of Pathology, Department of Surgical and Morphological Sciences, University of Insubria, Via O. Rossi, 9, 21100 Varese, Italy

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Fausto Sessa Section of Anatomic Pathology, CRIBI Biotechnology Center, Department of Molecular Medicine, Department of Pathology, Department of Surgical and Morphological Sciences, University of Insubria, Via O. Rossi, 9, 21100 Varese, Italy

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Stefano La Rosa Section of Anatomic Pathology, CRIBI Biotechnology Center, Department of Molecular Medicine, Department of Pathology, Department of Surgical and Morphological Sciences, University of Insubria, Via O. Rossi, 9, 21100 Varese, Italy

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Introduction High-grade (or poorly differentiated) gastroenteropancreatic (GEP) neuroendocrine carcinomas (NECs) are aggressive cancers with a high propensity for distant metastases. Like the more frequent pulmonary counterparts, they have

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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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a Ki-67 index ranging from 20 to 55% and those with a Ki-67 index above 55%. According to the current World Health Organization (WHO) 2010 Classification, neuroendocrine carcinoma (NEC) is defined by both a poorly differentiated morphology (large

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Mairéad G McNamara Department of Medical Oncology, The Christie NHS Foundation Trust, Manchester, UK
Division of Cancer Sciences, University of Manchester, Manchester, UK

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Jean-Yves Scoazec Department of Pathology, Gustave Roussy Cancer Campus, Villejuif, France
Université Paris Sud, Faculté de Médecine de Bicêtre, Le Kremlin-Bicêtre, France

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Thomas Walter Department of Gastroenterology and Medical Oncology, Edouard Herriot Hospital, Hospices Civils de Lyon, Lyon, France

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Introduction High grade neuroendocrine carcinomas (NECs) are usually defined by the combination of a poorly differentiated tumour cell morphology and evidence of high proliferative activity, evaluated either directly through the mitotic index

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O Hentic Pôle des Maladies de L'appareil Digestif, Service de Pathologie, Service de Radiologie, Service de Gastroentérologie–Pancréatologie

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P Hammel Pôle des Maladies de L'appareil Digestif, Service de Pathologie, Service de Radiologie, Service de Gastroentérologie–Pancréatologie

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A Couvelard Pôle des Maladies de L'appareil Digestif, Service de Pathologie, Service de Radiologie, Service de Gastroentérologie–Pancréatologie

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V Rebours Pôle des Maladies de L'appareil Digestif, Service de Pathologie, Service de Radiologie, Service de Gastroentérologie–Pancréatologie

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M Zappa Pôle des Maladies de L'appareil Digestif, Service de Pathologie, Service de Radiologie, Service de Gastroentérologie–Pancréatologie

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M Palazzo Pôle des Maladies de L'appareil Digestif, Service de Pathologie, Service de Radiologie, Service de Gastroentérologie–Pancréatologie

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F Maire Pôle des Maladies de L'appareil Digestif, Service de Pathologie, Service de Radiologie, Service de Gastroentérologie–Pancréatologie

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G Goujon Pôle des Maladies de L'appareil Digestif, Service de Pathologie, Service de Radiologie, Service de Gastroentérologie–Pancréatologie

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A Gillet Pôle des Maladies de L'appareil Digestif, Service de Pathologie, Service de Radiologie, Service de Gastroentérologie–Pancréatologie

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P Lévy Pôle des Maladies de L'appareil Digestif, Service de Pathologie, Service de Radiologie, Service de Gastroentérologie–Pancréatologie

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P Ruszniewski Pôle des Maladies de L'appareil Digestif, Service de Pathologie, Service de Radiologie, Service de Gastroentérologie–Pancréatologie

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condition due to aggressive and diffuse disease. These tumors are characterized by aggressive histological features (high Ki-67 index, extensive necrosis, and nuclear atypia) and are classified as neuroendocrine carcinomas (NECs) grade 3 according to the new

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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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-differentiated neuroendocrine tumours (NET G3) and poorly differentiated neuroendocrine carcinoma (NEC) ( WHO 2019 ). GEP-NEC have a particularly unfavourable prognosis, with median overall survival <1 year in advanced, treated cases and only 1 month if untreated ( Sorbye et

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

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Grace Kong Department of Molecular Imaging and Therapeutic Nuclear Medicine, Peter MacCallum Cancer Centre, Melbourne, Australia
Sir Peter MacCallum Department of Oncology, The University of Melbourne, Melbourne, Australia

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Simona Grozinsky-Glasberg Neuroendocrine Tumor Unit, ENETS Center of Excellence, Department of Endocrinology and Metabolism, Hadassah-Hebrew University Medical Center, Jerusalem, Israel

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of their proliferation index (G1–G3), whereas neuroendocrine carcinoma (NEC) category is used for poorly differentiated neoplasms. The high-grade GEP NEN G3 group (Ki-67 >20%) is therefore now separated into two groups: NET G3 and NEC ( Table 1 ) and

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Jessica L Geiger Division of Hematology/Oncology, Department of Internal Medicine, University of Pittsburgh Cancer Institute, Division of Anatomic Pathology, Department of Pathology, University of Pittsburgh Medical Center, Division of Endocrinology and Metabolism, Department of Internal Medicine, University of Pittsburgh Medical Center, Division of Molecular Genomic Pathology, Department of Pathology, University of Pittsburgh Medical Center, 5150 Centre Avenue, 5th Floor, Pittsburgh, Pennsylvania, 15232, USA

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Simion I Chiosea Division of Hematology/Oncology, Department of Internal Medicine, University of Pittsburgh Cancer Institute, Division of Anatomic Pathology, Department of Pathology, University of Pittsburgh Medical Center, Division of Endocrinology and Metabolism, Department of Internal Medicine, University of Pittsburgh Medical Center, Division of Molecular Genomic Pathology, Department of Pathology, University of Pittsburgh Medical Center, 5150 Centre Avenue, 5th Floor, Pittsburgh, Pennsylvania, 15232, USA

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Sue M Challinor Division of Hematology/Oncology, Department of Internal Medicine, University of Pittsburgh Cancer Institute, Division of Anatomic Pathology, Department of Pathology, University of Pittsburgh Medical Center, Division of Endocrinology and Metabolism, Department of Internal Medicine, University of Pittsburgh Medical Center, Division of Molecular Genomic Pathology, Department of Pathology, University of Pittsburgh Medical Center, 5150 Centre Avenue, 5th Floor, Pittsburgh, Pennsylvania, 15232, USA

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Marina N Nikiforova Division of Hematology/Oncology, Department of Internal Medicine, University of Pittsburgh Cancer Institute, Division of Anatomic Pathology, Department of Pathology, University of Pittsburgh Medical Center, Division of Endocrinology and Metabolism, Department of Internal Medicine, University of Pittsburgh Medical Center, Division of Molecular Genomic Pathology, Department of Pathology, University of Pittsburgh Medical Center, 5150 Centre Avenue, 5th Floor, Pittsburgh, Pennsylvania, 15232, USA

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Julie E Bauman Division of Hematology/Oncology, Department of Internal Medicine, University of Pittsburgh Cancer Institute, Division of Anatomic Pathology, Department of Pathology, University of Pittsburgh Medical Center, Division of Endocrinology and Metabolism, Department of Internal Medicine, University of Pittsburgh Medical Center, Division of Molecular Genomic Pathology, Department of Pathology, University of Pittsburgh Medical Center, 5150 Centre Avenue, 5th Floor, Pittsburgh, Pennsylvania, 15232, USA

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). Tumor formation occurs in the neuroendocrine organs where constitutively activated RET is expressed. Although neuroendocrine carcinoma (NEC) of the lung has been described in MEN 1 ( Farhandi et al . 1987 ), it has not been described in the MEN2

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