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Neuroendocrine Neoplasms (NENs) in Complex Genetic Disorders

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Polyendocrine Disorders and Endocrine Neoplastic Syndromes

Part of the book series: Endocrinology ((ENDOCR))

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Abstract

Neuroendocrine neoplasms (NENs) represent a group of neoplasms arising from the neuroendocrine cells of the diffuse endocrine system. The majority of gastrointestinal, pancreatic, lung, and thymic carcinoids are sporadic. However, some of the NEN may occur as part of multisystem autosomal-dominant inherited genetic disorders, such as the multiple endocrine neoplasia type 1 (MEN1) syndrome, von Hippel-Lindau disease (VHL), neurofibromatosis type 1 (NF-1), tuberous sclerosis complex (TSC), Mahvash syndrome, and the Pacak-Zhuang syndrome. Over the last three decades, the genetic basis of tumorigenesis in the context of these familial syndromes has been unraveled, thus providing clinicians with useful screening tools for affected families.

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Authors and Affiliations

  1. Department of Internal Medicine, Sector of Endocrinology, ENETS Center of Excellence for Neuroendocrine Tumours, Erasmus MC & Erasmus MC Cancer Center, Rotterdam, The Netherlands

    Wouter W. de Herder

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  1. Wouter W. de Herder
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Correspondence to Wouter W. de Herder .

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Editors and Affiliations

  1. Neuroendocrine Unit, University of Naples Federico II Neuroendocrine Unit, Naples, Italy

    Annamaria Colao

  2. Dept. Experimental Medicine, University of L'Aquila Dept. Experimental Medicine, L'Aquila, Italy

    Marie-Lise Jaffrain-Rea

  3. Department of Endocrinology, Centre Hospitalier Universitaire de Lièg Department of Endocrinology, Liège, Belgium

    Albert Beckers

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de Herder, W.W. (2019). Neuroendocrine Neoplasms (NENs) in Complex Genetic Disorders. In: Colao, A., Jaffrain-Rea, ML., Beckers, A. (eds) Polyendocrine Disorders and Endocrine Neoplastic Syndromes. Endocrinology. Springer, Cham. https://doi.org/10.1007/978-3-319-73082-0_16-1

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