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Krystallenia I Alexandraki Department of Surgery, Medical School, National and Kapodistrian University of Athens, Athens, Greece

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Gregory A Kaltsas Department of Propaedeutic Internal Medicine, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

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

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L , 2014 Lanreotide in metastatic enteropancreatic neuroendocrine tumors . New England Journal of Medicine 371 224 – 233 . ( https://doi.org/10.1056/NEJMoa1316158 ) 25014687 Cardoso Filho Fde A Feitosa RG Fechine CO Matos CM Cardoso AL

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Wenzel M Hackeng Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands

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Lodewijk A A Brosens Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands

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Koen M A Dreijerink Department of Pathology, University Medical Center Utrecht, Utrecht, The Netherlands
Department of Endocrinology, Amsterdam University Medical Centers, VU University Medical Center, Amsterdam, The Netherlands

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Introduction Insulinomas are neuroendocrine tumors that autonomously secrete insulin and usually arise within the pancreas. Insulinomas are uncommon tumors, with an incidence of 1–4 per million per year and are usually considered ‘benign’ or

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Francesca Marini Fondazione FIRMO Onlus, Italian Foundation for the Research on Bone Diseases, Florence, Italy

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Francesca Giusti Donatello Bone Clinic, Villa Donatello Hospital, Sesto Fiorentino, Italy

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Maria Luisa Brandi Fondazione FIRMO Onlus, Italian Foundation for the Research on Bone Diseases, Florence, Italy
Donatello Bone Clinic, Villa Donatello Hospital, Sesto Fiorentino, Italy

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Introduction Pancreatic neuroendocrine neoplasms (PanNENs) are rare tumors arising from the neuroendocrine cells of the pancreas, accounting for 1–3% of all types of pancreatic tumors ( Bevere et al. 2023 ). They can be classified into two

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Bence Sipos Department of Medical Oncology and Pneumology (Internal Medicine VIII), University Hospital Tubingen, Tübingen, Germany
ENETS Center of Excellence, University Hospital Tübingen, Tübingen, Germany

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Günter Klöppel Department of Pathology, Technical University Munich, Munich, Germany

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glucagon-producing neuroendocrine tumors. History and histology The first well-documented cases of GCHN were published in 2006 by Martin Anlauf and colleagues in an article on microadenomatosis of the endocrine pancreas in patients with and without

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Emanuel Christ Division of Endocrinology, Diabetology and Metabolism and Center of Endocrine Tumors, University Hospital of Basel, Basel, Switzerland

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Donato Iacovazzo Centre for Endocrinology, William Harvey Research Institute, Barts and London School of Medicine, Queen Mary University of London, London, United Kingdom

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Márta Korbonits Centre for Endocrinology, William Harvey Research Institute, Barts and London School of Medicine, Queen Mary University of London, London, United Kingdom

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

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2004 , Valente et al. 2018 ). In adults, most cases (approximately 90%) of EHH are secondary to a single insulinoma, a well-differentiated insulin-producing pancreatic neuroendocrine tumor. In about 5% of cases, EHH is secondary to an insulinoma

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Alaa Sada Department of Surgery, Division of Endocrinology, Division of Gastroenterology and Hepatology, Division of Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA

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Travis J McKenzie Department of Surgery, Division of Endocrinology, Division of Gastroenterology and Hepatology, Division of Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA

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Adrian Vella Department of Surgery, Division of Endocrinology, Division of Gastroenterology and Hepatology, Division of Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA

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Michael J Levy Department of Surgery, Division of Endocrinology, Division of Gastroenterology and Hepatology, Division of Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA

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Thorvardur R Halfdanarson Department of Surgery, Division of Endocrinology, Division of Gastroenterology and Hepatology, Division of Medical Oncology, Mayo Clinic, Rochester, Minnesota, USA

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Introduction In 1926, Dr William J Mayo operated on a patient with hypoglycemia induced by metastatic pancreatic neuroendocrine tumor (pNET) which led to the first description of insulinoma ( Wilder et al. 1927 ). Although the first

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Wouter W de Herder Department of Internal Medicine, Sector of Endocrinology, Erasmus MC & Erasmus MC Cancer Institute, ENETS Center of Excellence, Rotterdam, the Netherlands

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Günter Klöppel Department of Pathology, Technical University Munich, Munich, Germany

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neoplasms of the neuroendocrine cell system ( Pearse 1974 , de Herder et al. 2016 ). Since 2000, the islet cell tumors are referred to by the world health organization (WHO) as pancreatic neuroendocrine neoplasms (PanNENs) ( Rindi et al. 2022 , Capella

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Wouter W de Herder Department of Internal Medicine, Sector of Endocrinology, Erasmus MC & Erasmus MC Cancer Institute, ENETS Center of Excellence, GD Rotterdam, the Netherlands

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Günter Klöppel Department of Pathology, Technical University Munich, Trogerstrasse, Munich, Germany

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. In addition, neuroendocrine neoplasias, hyperplasias and other pathologies arising in the insulin- or glucagon-secreting beta and alpha cells, respectively, were discovered and characterized. Even in the last decade, new pathologies of these cells

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Christine Sempoux Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland

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Günter Klöppel Department of Pathology, Technical University Munich, Munich, Germany

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Journal of Physiology. Endocrinology and Metabolism 304 E711 – E723 . ( https://doi.org/10.1152/ajpendo.00304.2012 ) Orujov M Lai KK & Forse CL 2019 Concurrent adult-onset diffuse β-cell nesidioblastosis and pancreatic neuroendocrine tumor: A

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