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M T Barakat, K Meeran, and S R Bloom

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, Ioannis I Androulakis, Wouter W de Herder, and Ashley B Grossman

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, H Dibra, and C Thirlwell

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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Tim J Takkenkamp, Mathilde Jalving, Frederik J H Hoogwater, and Annemiek M E Walenkamp

Introduction NETs are tumours that arise from epithelial cells with both neurological and endocrinological functions and are most commonly located in the gastro-intestinal (GI) tract (

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Christodoulos P Pipinikas, Alison M Berner, Teresa Sposito, and Christina Thirlwell

), 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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Atsuko Kasajima, Marianne Pavel, Silvia Darb-Esfahani, Aurelia Noske, Albrecht Stenzinger, Hironobu Sasano, Manfred Dietel, Carsten Denkert, Christoph Röcken, Bertram Wiedenmann, and Wilko Weichert

Introduction With an incidence rate of about 2.5–5 cases per 100 000 person-years, gastroenteropancreatic neuroendocrine tumours (GEP-NET) are rather rare neoplasms when compared with adenocarcinomas of the same locations ( Modlin et al . 2008

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G A Kaltsas, D Papadogias, P Makras, and A B Grossman

Introduction Neuroendocrine tumours (NETs) constitute a heterogeneous group of tumours that originate from the diffuse endocrine system (DES). This system includes endocrine glands, such as the pituitary, the parathyroids and the

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Georgios K Dimitriadis, Martin O Weickert, Harpal S Randeva, Gregory Kaltsas, and Ashley Grossman

Introduction Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are considered to be relatively rare, with an estimated incidence of 3–5 cases per 100,000 inhabitants; however, recent data indicate that their prevalence is increasing

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Annemiek Walenkamp, Guillermo Crespo, Felipe Fierro Maya, Reidar Fossmark, Peter Igaz, Anja Rinke, Gianluca Tamagno, Giovanni Vitale, Kjell Öberg, and Tim Meyer

Introduction Neuroendocrine tumours (NETs) are diverse in their site of origin and clinical behaviour, ranging from highly aggressive small cell cancers of the lung to indolent, low-grade tumours of the small bowel ( Yao et al . 2008 a ). Symptoms

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Bristi Basu, Bhawna Sirohi, and Pippa Corrie

Introduction Malignant neuroendocrine tumours (NETs) are rare, comprising ∼2% of all malignant tumours diagnosed in the western world. They can arise from neuroendocrine cells anywhere in the body and form a spectrum of cancers that vary in