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Mohid S Khan Neuroendocrine Tumour Unit, Centre for Gastroenterology, Royal Free Hospital, 10th Floor, London NW3 2QG, UK

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Martyn E Caplin Neuroendocrine Tumour Unit, Centre for Gastroenterology, Royal Free Hospital, 10th Floor, London NW3 2QG, UK

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as a curative approach. Often neuroendocrine tumours (NETs) have metastases present at the time of diagnosis; therefore, curative resection is usually not possible, and when surgery is undertaken, it can be considered palliative in view of residual

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Christodoulos P Pipinikas Medical Genomics Laboratory, University College London Cancer Institute, London, UK

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Alison M Berner Medical Genomics Laboratory, University College London Cancer Institute, London, UK

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Teresa Sposito Medical Genomics Laboratory, University College London Cancer Institute, London, UK

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Christina Thirlwell Medical Genomics Laboratory, University College London Cancer Institute, London, UK
Neuroendocrine Tumour Unit and Department of Oncology, Royal Free Hospital, London, UK

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), most commonly liver metastases, 5-year survival is 16–46% ( Mignon et al. 2000 , Viúdez et al. 2016 , Batukbhai et al. 2019 ) ( Fig. 1A ). Figure 1 Overview of the multi-layered pancreatic neuroendocrine tumours (PanNET) complexity. (A

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Anela Blažević Department of Internal Medicine, Section Endocrinology, ENETS Centre of Excellence for Neuroendocrine Tumours, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, The Netherlands

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Wouter T Zandee Department of Internal Medicine, Section Endocrinology, ENETS Centre of Excellence for Neuroendocrine Tumours, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, The Netherlands

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Gaston J H Franssen Department of Surgery, ENETS Centre of Excellence for Neuroendocrine Tumours, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, The Netherlands

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Johannes Hofland Department of Internal Medicine, Section Endocrinology, ENETS Centre of Excellence for Neuroendocrine Tumours, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, The Netherlands

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Marie-Louise F van Velthuysen Department of Pathology, ENETS Centre of Excellence for Neuroendocrine Tumours, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, The Netherlands

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Leo J Hofland Department of Internal Medicine, Section Endocrinology, ENETS Centre of Excellence for Neuroendocrine Tumours, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, The Netherlands

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Richard A Feelders Department of Internal Medicine, Section Endocrinology, ENETS Centre of Excellence for Neuroendocrine Tumours, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, The Netherlands

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Wouter W de Herder Department of Internal Medicine, Section Endocrinology, ENETS Centre of Excellence for Neuroendocrine Tumours, Erasmus University Medical Center and Erasmus MC Cancer Institute, Rotterdam, The Netherlands

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Introduction Small intestinal neuroendocrine tumours (SI-NETs) are rare neoplasms with an incidence of approximately 0.2–1.2 per 100,000 individuals. However, due to the mostly indolent nature and increasing incidence, their prevalence is

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C Bouvier Neuroendocrine Tumour Unit, Royal Free Hospital, London NW3 2QG, UK.

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Nurse developments in the management of neuroendocrine tumours have changed significantly over the past three years. At the Royal Free Hospital we set up the nurse specialist role due to the expansion of patients being referred to the specialist unit, and the obvious need for a nursing input into their care. The nurse specialist can make a significant contribution within the context of a multidisciplinary team especially in the production of guidelines and policies to ensure and maintain high standards of practice, education for the patient, and the provision of expertise and security that the patient requires when diagnosed with a rare disease.

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R Srirajaskanthan
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G Dancey Neuroendocrine Tumour Unit, UCL Cancer Institute, Cancer Research UK and UCL Cancer Trials Centre, Department of Histopathology, Royal Free Hospital, London NW3 2QG, UK

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A Hackshaw Neuroendocrine Tumour Unit, UCL Cancer Institute, Cancer Research UK and UCL Cancer Trials Centre, Department of Histopathology, Royal Free Hospital, London NW3 2QG, UK

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T Luong Neuroendocrine Tumour Unit, UCL Cancer Institute, Cancer Research UK and UCL Cancer Trials Centre, Department of Histopathology, Royal Free Hospital, London NW3 2QG, UK

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M E Caplin
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T Meyer Neuroendocrine Tumour Unit, UCL Cancer Institute, Cancer Research UK and UCL Cancer Trials Centre, Department of Histopathology, Royal Free Hospital, London NW3 2QG, UK

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). Neuroendocrine tumours (NETs) are uncommon tumours that can occur in almost any organ and are thought to originate from neuroendocrine cells ( Caplin et al . 1998 , Modlin et al . 2008 ). The most common sites of origin is from gastroenteropancreatic tract

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Luohai Chen Neuroendocrine Tumour Unit, ENETS Centre of Excellence, Royal Free London NHS Foundation, London, UK
Department of Gastroenterology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, China
Institute for Liver and Digestive Disease, Royal Free Hospital, University College London, London, UK

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Gopinath Gnanasegaran Department of Nuclear Medicine, Royal Free London NHS Foundation, London, UK

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Dalvinder Mandair Neuroendocrine Tumour Unit, ENETS Centre of Excellence, Royal Free London NHS Foundation, London, UK

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Christos Toumpanakis Neuroendocrine Tumour Unit, ENETS Centre of Excellence, Royal Free London NHS Foundation, London, UK

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Martyn Caplin Neuroendocrine Tumour Unit, ENETS Centre of Excellence, Royal Free London NHS Foundation, London, UK

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Shaunak Navalkissoor Department of Nuclear Medicine, Royal Free London NHS Foundation, London, UK

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Introduction 177 Lutetium-DOTA 0 -Tyr 3 -octreotate ( 177 Lu-Dotatate) is a peptide receptor radionuclide therapy (PRRT) licensed for treatment in patients with advanced well-differentiated gastroenteropancreatic neuroendocrine tumours (NET

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D Mandair UCL Cancer Institute, University College London, London, UK
Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK

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C Vesely UCL Cancer Institute, University College London, London, UK

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L Ensell UCL Cancer Institute, University College London, London, UK

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H Lowe UCL Cancer Institute, University College London, London, UK

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V Spanswick UCL Cancer Institute, University College London, London, UK

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J A Hartley UCL Cancer Institute, University College London, London, UK

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M E Caplin Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK

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T Meyer UCL Cancer Institute, University College London, London, UK
Neuroendocrine Tumour Unit, Royal Free Hospital, London, UK

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limited by low rates of detection and isolation. CTCs have been identified in patients with metastatic neuroendocrine tumours (NETs) using the FDA-cleared CellSearch (Janssen Diagnostics) technology, a semi-automated platform that uses immunomagnetic

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M T Barakat Department of Metabolic Medicine, Division of Investigative Science, Imperial College London at Hammersmith Campus, Du Cane Road, London W12 ONN, UK.

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K Meeran Department of Metabolic Medicine, Division of Investigative Science, Imperial College London at Hammersmith Campus, Du Cane Road, London W12 ONN, UK.

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S R Bloom Department of Metabolic Medicine, Division of Investigative Science, Imperial College London at Hammersmith Campus, Du Cane Road, London W12 ONN, UK.

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Neuroendocrine tumours are a heterogeneous group including, for example, carcinoid, gastroenteropancreatic neuroendocrine tumours, pituitary tumours, medullary carcinoma of the thyroid and phaeochromocytomas. They have attracted much attention in recent years, both because they are relatively easy to palliate and because they have indicated the chronic effect of the particular hormone elevated. As neuroendocrine phenotypes became better understood, the definition of neuroendocrine cells changed and is now accepted as referring to cells with neurotransmitter, neuromodulator or neuropeptide hormone production, dense-core secretory granules, and the absence of axons and synapses. Neuroendocrine markers, particularly chromogranin A, are invaluable diagnostically. Study of several neuroendocrine tumours has revealed a genetic etiology, and techniques such as genetic screening have allowed risk stratification and prevention of morbidity in patients carrying the particular mutation. Pharmacological therapy for these often slow-growing tumours, e.g. with somatostatin analogues, has dramatically improved symptom control, and radiolabelled somatostatin analogues offer targeted therapy for metastatic or inoperable disease. In this review, the diagnosis and management of patients with carcinoid, gut neuroendocrine tumours, multiple endocrine neoplasia types 1 and 2, and isolated phaeochromocytoma are evaluated.

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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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Introduction Neuroendocrine tumours (NETs) are derived from cells that have the unique ability to synthesise, store and secrete a variety of metabolically active substances, peptides and amines, which can cause distinct clinical syndromes. These

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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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discovery of mutations in the novel tumour suppressor genes and chromatin remodellers ATRX/DAXX through exome sequencing represent significant progress in our understanding of neuroendocrine tumour (NET) development. NETs are a heterogenous group of

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