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Emmanouil Saloustros Section on Endocrinology and Genetics, Program on Developmental Endocrinology & Genetics (PDEGEN) & Pediatric Endocrinology Inter-institute Training Program, Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), National Institutes of Health (NIH), Bethesda, Maryland, USA

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Paraskevi Salpea Section on Endocrinology and Genetics, Program on Developmental Endocrinology & Genetics (PDEGEN) & Pediatric Endocrinology Inter-institute Training Program, Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), National Institutes of Health (NIH), Bethesda, Maryland, USA

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Matthew Starost Diagnostic and Research Services Branch, Division of Veterinary Resources (DVR), Office of Research Services (ORS), National Institutes of Health (NIH), Bethesda, Maryland, USA

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Sissi Liu Section on Endocrinology and Genetics, Program on Developmental Endocrinology & Genetics (PDEGEN) & Pediatric Endocrinology Inter-institute Training Program, Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), National Institutes of Health (NIH), Bethesda, Maryland, USA

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Fabio R Faucz Section on Endocrinology and Genetics, Program on Developmental Endocrinology & Genetics (PDEGEN) & Pediatric Endocrinology Inter-institute Training Program, Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), National Institutes of Health (NIH), Bethesda, Maryland, USA

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Edra London Section on Endocrinology and Genetics, Program on Developmental Endocrinology & Genetics (PDEGEN) & Pediatric Endocrinology Inter-institute Training Program, Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), National Institutes of Health (NIH), Bethesda, Maryland, USA

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Eva Szarek Section on Endocrinology and Genetics, Program on Developmental Endocrinology & Genetics (PDEGEN) & Pediatric Endocrinology Inter-institute Training Program, Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), National Institutes of Health (NIH), Bethesda, Maryland, USA

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Woo-Jin Song Department of Pediatrics, Metabolism Division, John Hopkins University, Baltimore, Maryland, USA

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Mehboob Hussain Department of Pediatrics, Metabolism Division, John Hopkins University, Baltimore, Maryland, USA

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Constantine A Stratakis Section on Endocrinology and Genetics, Program on Developmental Endocrinology & Genetics (PDEGEN) & Pediatric Endocrinology Inter-institute Training Program, Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD), National Institutes of Health (NIH), Bethesda, Maryland, USA

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(5%) also formed in the aged heterozygote ( Prkar1a +/− ) mouse. To better understand the role of PRKAR1A in the pancreas, we knocked out Prkar1a in pdx1 -expressing cells. Interestingly, these mice developed with high-penetrance malignant

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-16-0443
Volume/Issue:
Volume 24: Issue 1
Free access
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

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-17-0040
Volume/Issue:
Volume 24: Issue 8
Free access
Margaux Foulfoin Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de Gastroentérologie et d’Oncologie Médicale, Lyon, France

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Emmanuelle Graillot Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de Gastroentérologie et d’Oncologie Médicale, Lyon, France
University of Lyon, Université Lyon 1, Lyon, France

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Mustapha Adham University of Lyon, Université Lyon 1, Lyon, France
Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de chirurgie, Lyon, France

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Pascal Rousset University of Lyon, Université Lyon 1, Lyon, France
Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de radiologie, Lyon, France

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Julien Forestier Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de Gastroentérologie et d’Oncologie Médicale, Lyon, France

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Valérie Hervieu University of Lyon, Université Lyon 1, Lyon, France
Hospices Civils de Lyon, Hôpital Edouard Herriot, Service Central d’Anatomie et Cytologie Pathologiques, Lyon, France

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Philip Robinson Hospices Civils de Lyon, DRCI, Lyon, France

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Jean-Yves Scoazec Hospices Civils de Lyon, Hôpital Edouard Herriot, Service Central d’Anatomie et Cytologie Pathologiques, Lyon, France

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Catherine Lombard-Bohas Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de Gastroentérologie et d’Oncologie Médicale, Lyon, France

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Thomas Walter Hospices Civils de Lyon, Hôpital Edouard Herriot, Service de Gastroentérologie et d’Oncologie Médicale, Lyon, France
University of Lyon, Université Lyon 1, Lyon, France

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the first occurrence of metastases for metachronous disease: gender, age, ECOG performance status (PS), primary tumor location (head, body or tail of the pancreas), functional status, serum plasma levels of chromogranin A (CgA), serum alkaline

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-16-0464
Volume/Issue:
Volume 24: Issue 2
Free access
Kimberly Kamp
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Ronald A M Damhuis Department of Internal Medicine, Comprehensive Cancer Centre Netherlands, Sector of Endocrinology, Erasmus MC, S‐Gravendijkwal 230, 3015 CE Rotterdam, The Netherlands

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Richard A Feelders
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Wouter W de Herder
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gastrointestinal (GE) and bronchopulmonary tracts, and pancreas. In addition, these tumors can also be found in other more rare primary localizations such as ovaries, liver, and kidneys. NETs that originate from cells of the diffuse neuroendocrine system of the

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-11-0315
Volume/Issue:
Volume 19: Issue 1
Free access
Nele Garbrecht
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Martin Anlauf
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Anja Schmitt Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Tobias Henopp
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Bence Sipos
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Andreas Raffel Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Claus F Eisenberger Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Wolfram T Knoefel Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Marianne Pavel Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Christian Fottner Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Thomas J Musholt Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Anja Rinke Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Rudolf Arnold Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Uta Berndt Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Ursula Plöckinger Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Bertram Wiedenmann Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Holger Moch Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Philipp U Heitz Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Paul Komminoth Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany
Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Aurel Perren Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany
Department of Pathology, Department of Pathology, Department of General, Department of Gastroenterology and Endocrinology, Department of Endocrinology, Department of General and Abdominal Surgery, Department of Gastroenterology and Endocrinology, Department of Hepatology and Gastroenterology, Department of Pathology, Institute of Pathology, University of Kiel, Michaelisstr. 11, 24105 Kiel, Germany

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Günter Klöppel
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Introduction Neuroendocrine tumors (NETs) of the stomach, intestine, and pancreas are heterogeneous, as far as their morphology, function, and biology are concerned. The WHO classification therefore distinguishes the gastroenteropancreatic NETs

Article Type:
Research Article
DOI:
https://doi.org/10.1677/ERC-07-0157
Volume/Issue:
Volume 15: Issue 1
Free access
Ophélie De Rycke Department of Gastroenterology and Pancreatology, ENETS Centre of Excellence, Beaujon University Hospital, Clichy, France
Université de Paris, Centre de Recherche sur l’Inflammation, INSERM, Paris, France

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Thomas Walter Department of Gastro-enterology and oncology, Hospices Civils de Lyon, Edouard Herriot University Hospital, Lyon, France

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Marine Perrier Department of Hepato-Gastroenterology and Digestive Oncology, Robert Debré University Hospital, Reims, France

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Olivia Hentic Department of Gastroenterology and Pancreatology, ENETS Centre of Excellence, Beaujon University Hospital, Clichy, France

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Catherine Lombard-Bohas Department of Gastro-enterology and oncology, Hospices Civils de Lyon, Edouard Herriot University Hospital, Lyon, France

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Romain Coriat Department of Gastroenterology, Cochin University Hospital, Paris, France

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Guillaume Cadiot Department of Hepato-Gastroenterology and Digestive Oncology, Robert Debré University Hospital, Reims, France

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Anne Couvelard Université de Paris, Centre de Recherche sur l’Inflammation, INSERM, Paris, France
Department of Pathology, ENETS Centre of Excellence, Bichat/Beaujon University Hospital, Paris/Clichy, France

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Philippe Ruszniewski Department of Gastroenterology and Pancreatology, ENETS Centre of Excellence, Beaujon University Hospital, Clichy, France
Université de Paris, Centre de Recherche sur l’Inflammation, INSERM, Paris, France

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Jérôme Cros Université de Paris, Centre de Recherche sur l’Inflammation, INSERM, Paris, France
Department of Pathology, ENETS Centre of Excellence, Bichat/Beaujon University Hospital, Paris/Clichy, France

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Louis de Mestier Department of Gastroenterology and Pancreatology, ENETS Centre of Excellence, Beaujon University Hospital, Clichy, France
Université de Paris, Centre de Recherche sur l’Inflammation, INSERM, Paris, France

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Raj N Reidy-Lagunes D 2020 Exceptional responses after cessation of therapy With alkylating agents for pancreatic neuroendocrine tumors . Pancreas 49 e14 – e16 . ( https://doi.org/10.1097/MPA.0000000000001451 ) Zehir A Benayed R Shah

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-21-0034
Volume/Issue:
Volume 28: Issue 7
Restricted access
W W de Herder Department of Internal Medicine: Section of Endocrinology, Erasmus MC, Dr Molewaterplein 40, 3015 GD Rotterdam, The Netherlands. w.w.deherder@erasmusmc.nl

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E P Krenning Department of Internal Medicine: Section of Endocrinology, Erasmus MC, Dr Molewaterplein 40, 3015 GD Rotterdam, The Netherlands. w.w.deherder@erasmusmc.nl

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C H J Van Eijck Department of Internal Medicine: Section of Endocrinology, Erasmus MC, Dr Molewaterplein 40, 3015 GD Rotterdam, The Netherlands. w.w.deherder@erasmusmc.nl

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S W J Lamberts Department of Internal Medicine: Section of Endocrinology, Erasmus MC, Dr Molewaterplein 40, 3015 GD Rotterdam, The Netherlands. w.w.deherder@erasmusmc.nl

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Endocrine tumours of the gastrointestinal tract and pancreas may present at different disease stages with either hormonal or hormone-related symptoms/syndromes, or without hormonal symptoms. They may occur either sporadically or as part of hereditary syndromes. In the therapeutic approach to a patient with these tumours, excessive hormonal secretion and/or its effects should always be controlled first. Tumour-related deficiencies or disorders should also be corrected. Subsequently, control should be aimed at the tumour growth. Surgery is generally considered as first-line therapy for patients with localized disease, as it can be curative. However, in patients with metastatic disease the role of first-line surgery is not clearly established and other therapies should be considered, such as non-surgical cytoreductive therapies, biotherapy (with somatostatin analogues or interferon-alpha), embolization and chemoembolization of liver metastases, chemotherapy (with single or multiple dose regimens) and peptide receptor-targeted radiotherapy. The delicate balance of the use of the different therapeutical options in patients with endocrine tumours of the gastrointestinal tract and pancreas emphasizes the importance of team approach and team expertise.

Article Type:
Abstract
DOI:
https://doi.org/10.1677/erc.0.0110019
Volume/Issue:
Volume 11: Issue 1
Free access
Atsuko Kasajima Department of Pathology, Technical University Munich, Munich, Germany
Member of the German Cancer Consortium (DKTK), Germany

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

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, intestinum and lung, since it was coined by Oberndorfer in 1907 in his seminal work on the NETs of the ileum, and defined by Hamperl in 1937 for the NETs of the lung ( Hamperl 1937 ). Islet cell tumor is the term that denotes the NETs of the pancreas because

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-20-0122
Volume/Issue:
Volume 27: Issue 11
Free access
Taymeyah Al-Toubah Department of GI Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, USA

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Stefano Partelli Pancreas Translational & Clinical Research Center, Ospedale San Raffaele IRCCS, Milano, Italy
Università, Vita e Salute, Milan, Italy

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Mauro Cives Department of Biomedical Sciences and Human Oncology, University of Bari, Bari, Italy

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Valentina Andreasi Pancreas Translational & Clinical Research Center, Ospedale San Raffaele IRCCS, Milano, Italy

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Franco Silvestris Department of Biomedical Sciences and Human Oncology, University of Bari, Bari, Italy

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Massimo Falconi Pancreas Translational & Clinical Research Center, Ospedale San Raffaele IRCCS, Milano, Italy
Università, Vita e Salute, Milan, Italy

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Daniel A Anaya Department of GI Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, USA

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Jonathan Strosberg Department of GI Oncology, H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, USA

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Introduction Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are a heterogeneous group of tumors which include NETs of the gastrointestinal tract and pancreas. Tumor grade and differentiation are important prognostic factors. Low

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-18-0462
Volume/Issue:
Volume 26: Issue 4
Free access
G Rindi Department of Pathology and Laboratory Medicine, University of Parma,Via Gramsci 14, I-43100 Parma, Italy. guido.rindi@unipr.it

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C Bordi Department of Pathology and Laboratory Medicine, University of Parma,Via Gramsci 14, I-43100 Parma, Italy. guido.rindi@unipr.it

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Originating from cells of the diffuse endocrine system the endocrine tumours of the gut and the pancreatic tract are rare entities characterized by a common phenotypic aspect and producing several bioactive substances including growth factors. Two major categories are identified: well-differentiated and poorly differentiated tumours. The clinical behaviour varies ranging from benign to low grade malignant for well-differentiated tumours/carcinomas to high grade malignant for poorly differentiated carcinomas. The two major categories of well-differentiated and poorly differentiated tumours display distinct phenotypes and genetic backgrounds possibly supporting distinct histogenesis. Genetic abnormalities associated with either induction or progression of tumours may vary depending on the site of origin.

Article Type:
Abstract
Volume/Issue:
Volume 10: Issue 4
Free access