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Genetic Syndromes Associated with Neuroendocrine Tumours

  • Wouter W. de HerderEmail author
  • Gerlof D. Valk

Abstract

Neuroendocrime tumors (NETs) represent a group of neoplasms arising from the neuroendocrine cells of the diffuse endocrine system. The majority of gastrointestinal, pancreatic, lung, and thymic NET are sporadic. However, some of the NET may occur as part of multisystem autosomal dominant inherited genetic disorders, such as the MEN-1 syndrome, VHL, NF-1, and TSC. Over the last three decades, the genetic basis of tumorigenesis in the context of these familial syndromes has been elucidated, thus providing clinicians with useful screening tools for affected families.

Keywords

Tuberous Sclerosis Complex Clear Cell Renal Carcinoma Tuberous Sclerosis Complex Patient Familial Medullary Thyroid Carcinoma Retinal Angioma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.Department of Internal MedicineSector of Endocrinology, Erasmus MCRotterdamThe Netherlands
  2. 2.Department of Internal MedicineUniversity Medical Center UtrechtUtrechtThe Netherlands

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