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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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Maria Chiara Zatelli, Giuseppe Fanciulli, Pasqualino Malandrino, Valeria Ramundo, Antongiulio Faggiano, Annamaria Colao, and on behalf of NIKE Group

Introduction Neuroendocrine tumours (NETs) consist of a spectrum of malignancies that can arise from neuroendocrine cells throughout the body. Despite NETs having been considered rare for a long time, large epidemiological studies have reported that

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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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Eleonora Vitali, Valeria Cambiaghi, Alessandro Zerbi, Carlo Carnaghi, Piergiuseppe Colombo, Erika Peverelli, Anna Spada, Giovanna Mantovani, and Andrea G Lania

mediate most of the antisecretive and antiproliferative effects of SS ( Reubi & Schonbrunn 2013 ). Most neuroendocrine tumours (NETs) express SST2 and 5 that are targets for the long-acting SS analogues ( Papotti et al . 2002 , Reubi & Schonbrunn 2013

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Tobias Hofving, Viktor Sandblom, Yvonne Arvidsson, Emman Shubbar, Gülay Altiparmak, John Swanpalmer, Bilal Almobarak, Anna-Karin Elf, Viktor Johanson, Erik Elias, Erik Kristiansson, Eva Forssell-Aronsson, and Ola Nilsson

Introduction The 177 Lu-[DOTA 0 ,  Tyr 3 ]-octreotate therapy, or 177 Lu-octreotate therapy, is targeted against well-differentiated neuroendocrine tumours expressing somatostatin receptors. Following a promising retrospective study

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M Fraenkel, M Kim, A Faggiano, W W de Herder, G D Valk, and On behalf of the Knowledge NETwork

Introduction Neuroendocrine tumours (NETs) are heterogeneous neoplasms arising from different cells distributed in many organs and tissues that share a common neuroendocrine phenotype. NETs have been recognized as biologically different from

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Luohai Chen, Gopinath Gnanasegaran, Dalvinder Mandair, Christos Toumpanakis, Martyn Caplin, and Shaunak Navalkissoor

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

Open access

Martyn E Caplin, Marianne Pavel, Jarosław B Ćwikła, Alexandria T Phan, Markus Raderer, Eva Sedláčková, Guillaume Cadiot, Edward M Wolin, Jaume Capdevila, Lucy Wall, Guido Rindi, Alison Langley, Séverine Martinez, Edda Gomez-Panzani, Philippe Ruszniewski, and on behalf of the CLARINET Investigators

patients with metastatic grade 1 or 2 (proliferation index, Ki-67, up to 10%) somatostatin-receptor positive pancreatic, intestinal or of unknown primary origin neuroendocrine tumours (NETs) with prior stable disease (SD), irrespective of hepatic tumour

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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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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