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Clinical Approaches to the Management of Neuroendocrine Tumours

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International Manual of Oncology Practice

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Abstract

Neuroendocrine tumours (NET) are a rare and heterogeneous group of tumours. Over two-thirds originate from the gastrointestinal tract, and others include lung, breast, ovary and prostate (Modlin IM, Lye KD, Kidd M: Cancer 97(4):934–959, 2003). In 11–14% of cases the primary site is unknown [Hauso O, Gustafsson BI, Kidd M, Waldum HL, Drozdov I, Ak C et al: Cancer 113(10):2655–2664, 2008]. NET are classified according to their tissue origin, biochemical behavior, and prognosis (Ahlman H, Wängberg B, Jansson S, Friman S, Olausson M, Tylen U et al: Digestion 62:59–68, 2012). Functional tumours secrete bioactive peptides and may lead to the development of symptoms including flushing, wheezing, abdominal cramps, diarrhoea, blood pressure disturbance and tachycardia (Dong M, Phan T, Yao JC: Clin Cancer Res 18:1830–1836, 2012). Investigations include measurement of 24-h urinary 5-HIAA and chromogranin A. Management is dependent on symptoms at presentation, site of disease and tumour grade. Treatments include surgery for localised disease, ablative therapy, somatostatin analogues, chemotherapy and biological targeted therapy for advanced disease. Most patients present with advanced disease and in patients with metastatic disease median survival is around 24–27 months.

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Authors and Affiliations

  1. Velindre Cancer Centre, Cardiff, UK

    K. L. Yim & B. M. Thomas

  2. University Hospital Wales, Cardiff, UK

    A. Christian

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  1. K. L. Yim
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  2. B. M. Thomas
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Editors and Affiliations

  1. Division of Medical Oncology, Federal University of São Paulo (UNIFESP) & Hospital Israelita Albert Einstein, São Paulo, Brazil, Department of Biomedical Sciences and Medicine, University of Algarve, Faro, Portugal

    Ramon Andrade De Mello

  2. Oncology Department, Henry Dunant Hospital Center, Athens, Greece

    Giannis Mountzios

  3. Department of Biomedical Sciences and Medicine, Universidade do Algarve, Faro, Portugal

    Álvaro A. Tavares

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Yim, K.L., Thomas, B.M., Christian, A. (2019). Clinical Approaches to the Management of Neuroendocrine Tumours. In: De Mello, R., Mountzios, G., Tavares, Á. (eds) International Manual of Oncology Practice. Springer, Cham. https://doi.org/10.1007/978-3-030-16245-0_38

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