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Krystallenia I Alexandraki Department of Pathophysiology, Neuroendocrine Tumor Unit, Oxford Centre for Diabetes, National University of Athens, Greece

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Gregory A Kaltsas Department of Pathophysiology, Neuroendocrine Tumor Unit, Oxford Centre for Diabetes, National University of Athens, Greece

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Simona Grozinsky-Glasberg Department of Pathophysiology, Neuroendocrine Tumor Unit, Oxford Centre for Diabetes, National University of Athens, Greece

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Eleftherios Chatzellis Department of Pathophysiology, Neuroendocrine Tumor Unit, Oxford Centre for Diabetes, National University of Athens, Greece

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Ashley B Grossman Department of Pathophysiology, Neuroendocrine Tumor Unit, Oxford Centre for Diabetes, National University of Athens, Greece

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in the range of 2–5 cases/100 000/year ( Yao et al . 2008 , Niederle et al . 2010 , Fraenkel et al . 2012 ). While previous epidemiological data considered appendiceal NENs (aNENs) to be the most common GI-NENs, the overall percentage has

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-15-0310
Volume/Issue:
Volume 23: Issue 1
Free access
Nicole Panarelli Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York, USA

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Kathrin Tyryshkin Laboratory of Translational RNA Biology, Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada

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Justin Jong Mun Wong Laboratory of Translational RNA Biology, Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada

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Adrianna Majewski Laboratory of Translational RNA Biology, Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada

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Xiaojing Yang Laboratory of Translational RNA Biology, Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada

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Theresa Scognamiglio Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York, USA

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Michelle Kang Kim Center for Carcinoid and Neuroendocrine Tumors of Mount Sinai, Icahn School of Medicine at Mount Sinai, New York, New York, USA

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Kimberly Bogardus HHMI, Laboratory of RNA Molecular Biology, The Rockefeller University, New York, New York, USA

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Thomas Tuschl HHMI, Laboratory of RNA Molecular Biology, The Rockefeller University, New York, New York, USA

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Yao-Tseng Chen Department of Pathology and Laboratory Medicine, Weill Cornell Medicine, New York, New York, USA

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Neil Renwick Laboratory of Translational RNA Biology, Department of Pathology and Molecular Medicine, Queen’s University, Kingston, Ontario, Canada
HHMI, Laboratory of RNA Molecular Biology, The Rockefeller University, New York, New York, USA

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ileal and appendiceal NETs (comparison B) and rectal and pancreatic NETs (comparison C). We also compared miRNA expression differences between NETs from each anatomic site of origin (i.e. pancreas, ileum, appendix or rectum) and the remaining three GEP

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-18-0244
Volume/Issue:
Volume 26: Issue 1
Free access
Martin B Niederle
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Monika Hackl Division of General Surgery, Bundesanstalt ‘Statistik Österreich’ (Austria Statistics), Department of Clinical Pathology, Section of Endocrine Surgery, Department of Surgery, Medical University of Vienna, Währinger Gürtel 18–20, A-1090 Vienna, Austria

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Klaus Kaserer Division of General Surgery, Bundesanstalt ‘Statistik Österreich’ (Austria Statistics), Department of Clinical Pathology, Section of Endocrine Surgery, Department of Surgery, Medical University of Vienna, Währinger Gürtel 18–20, A-1090 Vienna, Austria

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Bruno Niederle
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(IQR: 22) and 59 (IQR: 27) years respectively ( P value significant only before correction for multiple testing: P <0.05, then non-significant). Patients with appendiceal NETs were significantly younger (median 38 (IQR: 45) years) than patients with

Article Type:
Research Article
DOI:
https://doi.org/10.1677/ERC-10-0152
Volume/Issue:
Volume 17: Issue 4
Free access
R Sutton Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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H E Doran Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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E M I Williams Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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J Vora Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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S Vinjamuri Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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J Evans Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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F Campbell Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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M G T Raraty Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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P Ghaneh Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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M Hartley Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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G J Poston Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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J P Neoptolemos Department of Surgery, University of Liverpool, Royal Liverpool University Hospital, Liverpool, UK. r.sutton@liv.ac.uk

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Many clinicians prefer to avoid surgery in patients with carcinoid neoplasia, because of its slow growth and relatively favourable prognosis. Nevertheless, the commonest cause of death in patients with carcinoid is advanced metastatic disease, and both clinical and epidemiological data indicate that the more effectively the disease is ablated, the more long-lasting the benefit. Multidisciplinary management of patients with carcinoid must consider inherited risk, possible multiple carcinoids and/or synchronous non-carcinoid cancer, and the use of a range of investigations that also evaluate the 10% of patients with carcinoid syndrome with or without valvular heart disease. Although primary size is correlated with the presence of nodal with or without liver metastases, carcinoid tumours <1 cm in diameter may be metastatic at presentation, particularly those arising within the small intestine. In the jejunum and ileum, resection of all sizes of carcinoid with local and regional nodes is preferred, to prevent nodal dissemination causing mesenteric ischaemia with or without infarction. Resection of nodal metastases should be undertaken in those with persistent or recurrent nodal disease if possible. Appendiceal and right colonic carcinoids are most effectively treated by right hemicolectomy with local and regional nodal clearance, as for adenocarcinoma. However, for most appendiceal carcinoids which are <1 cm in diameter and non-invasive, appendicectomy alone is sufficient. For appendiceal carcinoids 1-2 cm in diameter, histopathological assessment helps to determine the need for hemicolectomy. Liver resection has been followed by prolonged 5 year survival in several series and is recommended in appropriate patients to attempt cure or to debulk metastatic disease. Liver transplantation has had only qualified success in highly selected patients without extra-hepatic disease in whom other therapies have failed.

Article Type:
Abstract
Volume/Issue:
Volume 10: Issue 4
Free access
Francesco Panzuto
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Silvia Nasoni
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Massimo Falconi
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Vito Domenico Corleto
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Gabriele Capurso
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Sara Cassetta
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Michela Di Fonzo
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Valentina Tornatore
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Massimo Milione
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Stefano Angeletti
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Maria Sofia Cattaruzza
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Vincenzo Ziparo
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Cesare Bordi
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Paolo Pederzoli
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Gianfranco Delle Fave
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of patients with appendiceal tumors was excluded from the risk factors analysis; it is well known that these tumors are usually characterized by a low risk of metastases and a relatively benign behavior ( Sutton et al. 2003 ). In fact, even in this

Article Type:
Research Article
DOI:
https://doi.org/10.1677/erc.1.01017
Volume/Issue:
Volume 12: Issue 4
Free access
Yvonne Arvidsson Lundberg Laboratory for Cancer Research, Lundberg Laboratory for Cancer Research, Department of Biochemistry, Department of Pathology

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Ellinor Andersson Lundberg Laboratory for Cancer Research, Lundberg Laboratory for Cancer Research, Department of Biochemistry, Department of Pathology

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Anders Bergström Lundberg Laboratory for Cancer Research, Lundberg Laboratory for Cancer Research, Department of Biochemistry, Department of Pathology

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Mattias K Andersson Lundberg Laboratory for Cancer Research, Lundberg Laboratory for Cancer Research, Department of Biochemistry, Department of Pathology

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Gülay Altiparmak Lundberg Laboratory for Cancer Research, Lundberg Laboratory for Cancer Research, Department of Biochemistry, Department of Pathology

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Ann-Christin Illerskog Lundberg Laboratory for Cancer Research, Lundberg Laboratory for Cancer Research, Department of Biochemistry, Department of Pathology

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Håkan Ahlman Lundberg Laboratory for Cancer Research, Lundberg Laboratory for Cancer Research, Department of Biochemistry, Department of Pathology

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Darima Lamazhapova Lundberg Laboratory for Cancer Research, Lundberg Laboratory for Cancer Research, Department of Biochemistry, Department of Pathology
Lundberg Laboratory for Cancer Research, Lundberg Laboratory for Cancer Research, Department of Biochemistry, Department of Pathology

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Ola Nilsson Lundberg Laboratory for Cancer Research, Lundberg Laboratory for Cancer Research, Department of Biochemistry, Department of Pathology

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carcinoids (7 enterochromaffin-like (ECL) cell tumours (benign) in chronic atrophic gastritis, 4 sporadic ECL cell tumours (malignant)), small intestinal (ileal) carcinoids (32 enterochromaffin cell tumours (malignant)), appendiceal carcinoids (18 classical

Article Type:
Research Article
DOI:
https://doi.org/10.1677/ERC-07-0145
Volume/Issue:
Volume 15: Issue 2
Free access
Sarah B Bateni Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
University of Alabama at Birmingham, Department of Surgery, Division of Surgical Oncology, Birmingham, Alabama, USA

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Natalie G Coburn Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
Susan Leslie Clinic for Neuroendocrine Tumors, Odette Cancer Centre - Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada
Clinical evaluative sciences, Sunnybrook Research Institute, Toronto, Ontario, Canada

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Calvin Law Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
Susan Leslie Clinic for Neuroendocrine Tumors, Odette Cancer Centre - Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada

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Simron Singh Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
Susan Leslie Clinic for Neuroendocrine Tumors, Odette Cancer Centre - Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada
Clinical evaluative sciences, Sunnybrook Research Institute, Toronto, Ontario, Canada

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Sten Myrehaug Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
Susan Leslie Clinic for Neuroendocrine Tumors, Odette Cancer Centre - Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada
Clinical evaluative sciences, Sunnybrook Research Institute, Toronto, Ontario, Canada

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Angela Assal Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
Susan Leslie Clinic for Neuroendocrine Tumors, Odette Cancer Centre - Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada
Clinical evaluative sciences, Sunnybrook Research Institute, Toronto, Ontario, Canada

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Julie Hallet Temerty Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada
Susan Leslie Clinic for Neuroendocrine Tumors, Odette Cancer Centre - Sunnybrook Health Sciences Centre, Toronto, Ontario, Canada
Clinical evaluative sciences, Sunnybrook Research Institute, Toronto, Ontario, Canada

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al. 2019 ). Study cohort Patients ≥ 20 years of age diagnosed with a gastrointestinal (gastric, small intestine, colon, rectal, or appendiceal), pancreatic, or lung primary NET between January 1, 2000, and October 31, 2016, were identified in

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-22-0337
Volume/Issue:
Volume 30: Issue 8
Restricted access
M Fraenkel
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M Kim Endocrinology, Mount Sinai Medical Center, Department of Clinical Medicine and Surgery, Endocrinology, Erasmus MC, Department of Internal Medicine, Soroka University Medical Center and the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheeba, Israel

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A Faggiano Endocrinology, Mount Sinai Medical Center, Department of Clinical Medicine and Surgery, Endocrinology, Erasmus MC, Department of Internal Medicine, Soroka University Medical Center and the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheeba, Israel
Endocrinology, Mount Sinai Medical Center, Department of Clinical Medicine and Surgery, Endocrinology, Erasmus MC, Department of Internal Medicine, Soroka University Medical Center and the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheeba, Israel

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W W de Herder Endocrinology, Mount Sinai Medical Center, Department of Clinical Medicine and Surgery, Endocrinology, Erasmus MC, Department of Internal Medicine, Soroka University Medical Center and the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheeba, Israel

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G D Valk Endocrinology, Mount Sinai Medical Center, Department of Clinical Medicine and Surgery, Endocrinology, Erasmus MC, Department of Internal Medicine, Soroka University Medical Center and the Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheeba, Israel

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On behalf of the Knowledge NETwork
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( Niederle et al . 2010 ; Fig. 2 A). Figure 2 Most recent incidence rates in various countries for six types of NET: (A) gastric, (B) small intestine, (C) pancreatic, (D) colon, (E) rectal, and (F) appendiceal NETs. Data for the various countries are from

Article Type:
Review Article
DOI:
https://doi.org/10.1530/ERC-13-0125
Volume/Issue:
Volume 21: Issue 3
Free access
Ulrich-Frank Pape Department of Hepatology and Gastroenterology, Institute for Social Medicine, Institute for Pathology, Department of Gastroenterology, Charité, Campus Virchow Klinikum

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Uta Berndt Department of Hepatology and Gastroenterology, Institute for Social Medicine, Institute for Pathology, Department of Gastroenterology, Charité, Campus Virchow Klinikum

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Jacqueline Müller-Nordhorn Department of Hepatology and Gastroenterology, Institute for Social Medicine, Institute for Pathology, Department of Gastroenterology, Charité, Campus Virchow Klinikum

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Michael Böhmig Department of Hepatology and Gastroenterology, Institute for Social Medicine, Institute for Pathology, Department of Gastroenterology, Charité, Campus Virchow Klinikum
Department of Hepatology and Gastroenterology, Institute for Social Medicine, Institute for Pathology, Department of Gastroenterology, Charité, Campus Virchow Klinikum

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Stephanie Roll Department of Hepatology and Gastroenterology, Institute for Social Medicine, Institute for Pathology, Department of Gastroenterology, Charité, Campus Virchow Klinikum

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Martin Koch Department of Hepatology and Gastroenterology, Institute for Social Medicine, Institute for Pathology, Department of Gastroenterology, Charité, Campus Virchow Klinikum

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Stefan N Willich Department of Hepatology and Gastroenterology, Institute for Social Medicine, Institute for Pathology, Department of Gastroenterology, Charité, Campus Virchow Klinikum

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Bertram Wiedenmann Department of Hepatology and Gastroenterology, Institute for Social Medicine, Institute for Pathology, Department of Gastroenterology, Charité, Campus Virchow Klinikum

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mid- (i.e. jejunal, ileal, appendiceal, caecal, ascending and right transverse colonic) and hindgut NET (i.e. left transverse colonic to rectal). This classification proved to be of only limited value because it is too inaccurate ( Klöppel et al

Article Type:
Research Article
DOI:
https://doi.org/10.1677/ERC-08-0017
Volume/Issue:
Volume 15: Issue 4
Free access
A Karpathakis University College London Cancer Institute, Neuroendocrine Tumour Unit, 72 Huntley Street, London WC1E 6BT, UK
University College London Cancer Institute, Neuroendocrine Tumour Unit, 72 Huntley Street, London WC1E 6BT, UK

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H Dibra University College London Cancer Institute, Neuroendocrine Tumour Unit, 72 Huntley Street, London WC1E 6BT, UK

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C Thirlwell University College London Cancer Institute, Neuroendocrine Tumour Unit, 72 Huntley Street, London WC1E 6BT, UK
University College London Cancer Institute, Neuroendocrine Tumour Unit, 72 Huntley Street, London WC1E 6BT, UK

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al . (2003 a ) HPM 0% appendiceal HPM NCI-H727 and HTB-119 Zhang et al . (2006) HPM 84.8% metastatic Zhang et al . (2006) HPM 60.6% non-metastatic Zhang et al . (2006) RASSF1 HPM

Article Type:
Research Article
DOI:
https://doi.org/10.1530/ERC-12-0338
Volume/Issue:
Volume 20: Issue 3
Free access