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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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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
Department of Pathology, Tohoku University Graduate School of Medicine, Sendai, Japan
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National Hospital Organization, Sendai Medical Center, Sendai, Japan
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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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-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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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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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
Division of Cancer Sciences, University of Manchester, Manchester, UK
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Université Paris Sud, Faculté de Médecine de Bicêtre, Le Kremlin-Bicêtre, 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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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
Department of Oncology, Haukeland University Hospital, Bergen, Norway
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Department of Clinical and Molecular Medicine, Faculty of Medicine and Health Sciences, Norwegian University of Science and Technology, Trondheim, Norway
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Department of Oncology, Haukeland University Hospital, Bergen, Norway
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Department of Medical Radiation Physics, Lund University, Lund, Sweden
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Department of Oncology, St.Olavs Hospital, Trondheim, Norway
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Department of Clinical Medicine, Faculty of Health Sciences, University of Southern Denmark, Odense, Denmark
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Department of Clinical Science, University of Bergen, Bergen, Norway
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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
Department of Clinical Sciences, University of Bergen, Bergen, Norway
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Sir Peter MacCallum Department of Oncology, The University of Melbourne, Melbourne, Australia
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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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). 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