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Hanbaro Kim, Ki Byung Song, Dae Wook Hwang, Jae Hoon Lee, Shadi Alshammary, and Song Cheol Kim

Introduction Pancreatic neuroendocrine tumors (PNETs), also known as islet cell tumors, are rare neoplasms arising from the endocrine pancreas, with a reported incidence of <1 per 100,000 persons per year ( 1 , 2 ). However, the incidence of

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Qi Zhang, Hongshan Wang, Yanhong Xie, Suming Huang, Ke Chen, Botian Ye, Yupeng Yang, Jie Sun, Hongyong He, Fenglin Liu, Zhenbin Shen, Weidong Chen, Kuntang Shen, Yuan Ji, and Yihong Sun

activity ( 1 ). The prognosis of high-grade GEP-NENs, also known as grade 3 GEP-NENs, was much worse than that of grade 1 neuroendocrine tumors (G1 NET) and grade 2 neuroendocrine tumors (G2 NET) ( 2 , 3 , 4 ). However, the 2010 WHO classification was

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Matteo Scopel, Eugenio De Carlo, Francesca Bergamo, Sabina Murgioni, Riccardo Carandina, Anna Rita Cervino, Marta Burei, Federica Vianello, Vittorina Zagonel, Matteo Fassan, and Roberto Vettor

Introduction Neuroendocrine tumors (NETs) are a heterogeneous family of neoplasms that originate from cells belonging to the widespread neuroendocrine system. These cells appear to be ubiquitous in the human body, but the main sites are the

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Katherine Van Loon, Li Zhang, Jennifer Keiser, Cendy Carrasco, Katherine Glass, Maria-Teresa Ramirez, Sarah Bobiak, Eric K Nakakura, Alan P Venook, Manisha H Shah, and Emily K Bergsland

Introduction Neuroendocrine tumors (NETs) arise from the many cells of the diffuse endocrine system, which possess unique functions and the potential for hormone production. As such, NETs represent a heterogeneous group of tumors with variable

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Elham Barazeghi, Per Hellman, Gunnar Westin, and Peter Stålberg

Introduction Small intestinal neuroendocrine tumors (SI-NETs) arise from enterochromaffin cells in the gastrointestinal tract, which are small and slow-growing (Ki67 proliferation index is often <2%) tumors with annual incidence of 1 per 100

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Olof Joakim Pettersson, Katarzyna Fröss-Baron, Joakim Crona, and Anders Sundin

Introduction Gastroenteropancreatic neuroendocrine tumors comprise a variety of tumors, originating from stem cells in the gastrointestinal canal and pancreatic islets. An incidence of approximately 5 per 100,000 person-years is often

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R C S van Adrichem, L J Hofland, R A Feelders, M C De Martino, P M van Koetsveld, C H J van Eijck, R R de Krijger, D M Sprij-Mooij, J A M J L Janssen, and W W de Herder

Introduction Gastroenteropancreatic neuroendocrine tumors (GEP NETs) are rare and heterogeneous tumors which may vary according to their biological, functional, and clinical behavior (1) . Chromogranin A (CgA) and the Ki-67 proliferation index are

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Kjell Oberg, Eric Krenning, Anders Sundin, Lisa Bodei, Mark Kidd, Margot Tesselaar, Valentina Ambrosini, Richard P Baum, Matthew Kulke, Marianne Pavel, Jaroslaw Cwikla, Ignat Drozdov, Massimo Falconi, Nicola Fazio, Andrea Frilling, Robert Jensen, Klaus Koopmans, Tiny Korse, Dik Kwekkeboom, Helmut Maecke, Giovanni Paganelli, Ramon Salazar, Stefano Severi, Jonathan Strosberg, Vikas Prasad, Aldo Scarpa, Ashley Grossman, Annemeik Walenkamp, Mauro Cives, Irene Virgolini, Andreas Kjaer, and Irvin M Modlin

indicators of tumor activity, but as this group of lesions represents a minority of NENs (<3–5%), its broad utility is limited. CgA is a constitutive product of the neuroendocrine cell secretory granule and is measurable in serum or plasma. It has been

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Kazhan Mollazadegan, Britt Skogseid, Johan Botling, Tobias Åkerström, Barbro Eriksson, Staffan Welin, Anders Sundin, and Joakim Crona

grading of panNENs ( 3 ), high-grade neoplasms were separated into two categories: well-differentiated (WD) pancreatic neuroendocrine tumor grade 3 (panNET-G3) and poorly differentiated (PD) NEC ( 4 ). PanNET-G3 frequently harbors mutations in MEN1 and

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Anela Blažević, Anand M Iyer, Marie-Louise F van Velthuysen, Johannes Hofland, Peter M van Koestveld, Gaston J H Franssen, Richard A Feelders, Marina Zajec, Theo M Luider, Wouter W de Herder, and Leo J Hofland

Introduction Small intestinal neuroendocrine tumors (SI-NETs) are accompanied by specific clinical pathology, most notable carcinoid syndrome and fibrotic complications such as carcinoid heart disease and mesenteric fibrosis (MF) ( 1 ). There