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Maria P Yavropoulou Endocrinοlogy Unit, 1st Department of Propaedeutic Internal Medicine, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Marina Tsoli Endocrinοlogy Unit, 1st Department of Propaedeutic Internal Medicine, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Konstantinos Barkas Department of Neurosurgery, General Hospital of Nikaia-Peiraia, Agios Panteleimon, Athens, Greece

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Gregory Kaltsas Endocrinοlogy Unit, 1st Department of Propaedeutic Internal Medicine, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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Ashley Grossman Centre for Endocrinology, Barts and the London School of Medicine, Queen Mary University of London, London, UK
Oxford Centre for Diabetes, Endocrinology and Metabolism, University of Oxford, Oxford, UK

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incidentally diagnosed during imaging investigations performed for unrelated purposes (pituitary incidentalomas, PIs) ( Scangas & Laws 2014 ). Recently, pituitary adenomas have been suggested to be renamed pituitary neuroendocrine tumours (pitNETs) ( Asa et al

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Roberto Valente Digestive and Liver Disease Unit, Sant’ Andrea Hospital, Sapienza University of Rome, Rome, Italy

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Alastair J Hayes Department of General Surgery, Royal Infirmary of Edinburgh, Edinburgh, UK

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Sven-Petter Haugvik Department of Hepato-Pancreato-Biliary Surgery, Oslo University Hospital, Oslo, Norway

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Per Hedenström Unit of Gastroenterology, Department of Medicine, Sahlgrenska University Hospital, Gothenburg, Sweden

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Darko Siuka Department of Gastroenterology, University Medical Centre Ljubljana, Ljubljana, Slovenia

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Emilie Korsæth Department of Hepato-Pancreato-Biliary Surgery, Oslo University Hospital, Oslo, Norway

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Daniel Kämmerer Department of General and Visceral Surgery, Zentralklinik Bad Berka, Bad Berka, Germany

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Stuart M Robinson Department of Hepatopancreatobiliary and Transplantation Surgery, The Freeman Hospital, Newcastle upon Tyne, UK

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Patrick Maisonneuve Division of Epidemiology and Biostatistics, European Institute of Oncology, Milan, Italy

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Gianfranco Delle Fave Digestive and Liver Disease Unit, Sant’ Andrea Hospital, Sapienza University of Rome, Rome, Italy

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Bjorn Lindkvist Unit of Gastroenterology, Department of Medicine, Sahlgrenska University Hospital, Gothenburg, Sweden

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Gabriele Capurso Digestive and Liver Disease Unit, Sant’ Andrea Hospital, Sapienza University of Rome, Rome, Italy

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Introduction Pancreatic neuroendocrine neoplasms (PNENs) are a group of tumors which originate from endocrine cells within the pancreas gland. PNENs have heterogeneous clinical behavior owing to their hormone functional status, cellular

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Gregory Kaltsas
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Ioannis I Androulakis
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Wouter W de Herder Endocrine Unit, Department of Pathophysiology, National University of Athens, Department of Internal Medicine, Sector of Endocrinology, Department of Endocrinology, St Bartholomew's Hospital, Mikras Asias 75, 11527 Athens, Greece

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Ashley B Grossman Endocrine Unit, Department of Pathophysiology, National University of Athens, Department of Internal Medicine, Sector of Endocrinology, Department of Endocrinology, St Bartholomew's Hospital, Mikras Asias 75, 11527 Athens, Greece

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L 2003 Paraneoplastic endocrine syndromes: a review . Endocrine Pathology 14 303 – 317 . Deleu D De Geeter F 1991 Neurological manifestations of neuroendocrine neoplasms of the larynx . ORL: Journal for Otorhinolaryngology and its

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Katarina Edfeldt Departments of Surgical Sciences, Medical Sciences, Uppsala University Hospital, Uppsala University, Entrance 70, 3 tr, SE-75185 Uppsala, Sweden

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Tanveer Ahmad Departments of Surgical Sciences, Medical Sciences, Uppsala University Hospital, Uppsala University, Entrance 70, 3 tr, SE-75185 Uppsala, Sweden

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Göran Åkerström Departments of Surgical Sciences, Medical Sciences, Uppsala University Hospital, Uppsala University, Entrance 70, 3 tr, SE-75185 Uppsala, Sweden

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Eva Tiensuu Janson Departments of Surgical Sciences, Medical Sciences, Uppsala University Hospital, Uppsala University, Entrance 70, 3 tr, SE-75185 Uppsala, Sweden

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Per Hellman Departments of Surgical Sciences, Medical Sciences, Uppsala University Hospital, Uppsala University, Entrance 70, 3 tr, SE-75185 Uppsala, Sweden

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Peter Stålberg Departments of Surgical Sciences, Medical Sciences, Uppsala University Hospital, Uppsala University, Entrance 70, 3 tr, SE-75185 Uppsala, Sweden

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Peyman Björklund Departments of Surgical Sciences, Medical Sciences, Uppsala University Hospital, Uppsala University, Entrance 70, 3 tr, SE-75185 Uppsala, Sweden

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Gunnar Westin Departments of Surgical Sciences, Medical Sciences, Uppsala University Hospital, Uppsala University, Entrance 70, 3 tr, SE-75185 Uppsala, Sweden

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intestine, especially in the distal ileum. According to the WHO classification system, from year 2010, SI-NETs are divided into three grades ( Bosman et al . 2010 ); Grade 1 Neuroendocrine neoplasm (Ki67 <3%), Grade 2 Neuroendocrine neoplasm (Ki67 3

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Daniela Molè
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Teresa Gagliano
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Erica Gentilin
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Federico Tagliati
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Claudio Pasquali Section of Endocrinology, Department of Medical and Surgical Sciences, Section of Clinical Surgery, Department of Biomedical Sciences and Advanced Therapies, University of Ferrara, Via Savonarola 9, 44121 Ferrara, Italy

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Maria Rosaria Ambrosio
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Giancarlo Pansini Section of Endocrinology, Department of Medical and Surgical Sciences, Section of Clinical Surgery, Department of Biomedical Sciences and Advanced Therapies, University of Ferrara, Via Savonarola 9, 44121 Ferrara, Italy

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Ettore C degli Uberti
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Maria Chiara Zatelli
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Introduction Pancreatic neuroendocrine neoplasms (PNN) account for <3% of pancreatic tumors ( Ehehalt et al . 2009 ). Current therapy is complete surgical resection ( Fendrich et al . 2006 ), which is however achieved in the minority of cases

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Uriel Clemente-Gutierrez Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Carolina R C Pieterman Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands

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Michael S Lui Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Thomas Szabo Yamashita Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Andrés Tame-Elorduy Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Bernice L Huang Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Aditya S Shirali Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Derek J Erstad Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Jeffrey E Lee Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Sarah B Fisher Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Paul H Graham Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Elizabeth G Grubbs Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Steven G Waguespack Department of Endocrine Neoplasia and Hormonal Disorders, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Chaan S Ng Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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Nancy Perrier Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas, USA

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nodules showed neoplasm of uncertain malignant potential. Figure 2 Follow-up of nodular adrenal lesions. *Three nodules in two patients (one patient had bilateral neoplasm with uncertain malignant potential). pNET, pancreatic neuroendocrine tumor

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Kjell Oberg Department of Endocrine Oncology, Department of Medical Sciences, University Hospital, Entrance 78, SE-751 85 Uppsala, Sweden
Department of Endocrine Oncology, Department of Medical Sciences, University Hospital, Entrance 78, SE-751 85 Uppsala, Sweden

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classification and the pathology of gastro-entero-pancreatic neuroendocrine neoplasms is discussed by Prof. Günter Klöppel ( Klöppel 2011 ). A number of biomarkers has been developed during the last decades based on development of the immunoassays for various gut

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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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al . 2016 , McNamara et al . 2019 ). There has been recent progress in the pathological diagnosis of high grade neuroendocrine neoplasms (NENs) ( Lloyd et al . 2017 , Rindi et al . 2018 a ) and in the description of their molecular and immune

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Massimo Milione 1st Pathology Division, Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

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Patrick Maisonneuve Division of Epidemiology and Biostatistics, European Institute of Oncology (IEO), Milan, Italy

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Alessio Pellegrinelli 1st Pathology Division, Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

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Federica Grillo Pathology Unit, Department of Surgical Sciences and Integrated Diagnostics (DISC), University of Genova and Policlinico San Martino, Genova, Italy

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Luca Albarello Pathology Unit, IRCCS San Raffaele Scientifica Institute, Milan, Italy

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Paola Spaggiari Cancer Center Humanitas, Milan, Italy

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Alessandro Vanoli Fondazione IRCCS Policlinico San Matteo and Department of Molecular Medicine, University of Pavia, Pavia, Italy

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Giovanna Tagliabue Lombardy Cancer Registry, Varese Province Cancer Registry Unit, Department of Research, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

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Eleonora Pisa Division of Pathology, European Institute of Oncology (IEO), Milan, Italy

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Luca Messerini Diagnostic and Molecular Pathology, Department of Experimental and Clinical Medicine, University of Florence, Firenze, Italy

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Giovanni Centonze Department of Experimental Oncology and Molecular Medicine, Unit of Tumor Genomics, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
Clinical Research Lab (CRAB), Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy

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Frediano Inzani Anatomic Pathology Unit, Fondazione Policlinico Universitario A. Gemelli, Rome, Italy

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Aldo Scarpa ARC-Net Research Centre and Department of Diagnostics and Public Health-Section of Pathology, University and Hospital Trust of Verona, Verona, Italy

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Mauro Papotti Department of Oncology, University of Turin, Turin, Italy

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Marco Volante Department of Oncology, University of Turin, Turin, Italy

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Fausto Sessa Department of Medicine and Surgery, University of Insubria, Varese, Italy

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Nicola Fazio Gastrointestinal Medical Oncology and Neuroendocrine Tumors Unit, European Institute of Oncology (IEO), Milan, Italy

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Giancarlo Pruneri 1st Pathology Division, Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
University of Milan, School of Medicine, Milan, Italy

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Guido Rindi Institute of Anatomic Pathology, Università Cattolica del Sacro Cuore-Fondazione Policlinico Universitario A. Gemelli, Rome, Italy

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Enrico Solcia Fondazione IRCCS Policlinico San Matteo and Department of Molecular Medicine, University of Pavia, Pavia, Italy

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Stefano La Rosa Service of Clinical Pathology, Lausanne University Hospital, Institute of Pathology, Lausanne, Switzerland

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Carlo Capella Department of Medicine and Surgery, University of Insubria, Varese, Italy

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Introduction The coexistence of neuroendocrine and non-neuroendocrine components in the same neoplasm is a rare but well-known phenomenon in digestive system tumors. The 2010 World Health Organization (WHO) classification of tumors of the

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Mei Dong Department of Gastrointestinal Medical Oncology, Unit 426, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030, USA

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James C Yao Department of Gastrointestinal Medical Oncology, Unit 426, The University of Texas M. D. Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, Texas 77030, USA

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Bronchial carcinoids Bronchial carcinoid (BC), the second most common carcinoid tumor after gastrointestinal carcinoids, accounts for 20–30 percent of all cases of well-differentiated neuroendocrine tumors. With an incidence rate of 1.35 per 1 00

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