title: Genetics and Epigenetics of Pancreatic Neuroendocrine Tumors and Pulmonary Carcinoids.
abstract: In this chapter, we give an overview of the genetic and epigenetic background of neuroendocrine tumors (NETs), in particular pancreatic and pulmonary NETs. Studying the mechanism of disease of the inherited syndromes that feature NETs has provided valuable insights that have revolutionized the therapeutic options for these tumor types: both inhibition of mTOR (mammalian target of rapamycin) signaling and inhibition of angiogenesis have become standard treatments. Although sporadic NETs harbor relatively few somatic gene mutations, these somatic mutations often affect genes that encode epigenetic regulators. Restoring the aberrant epigenetic characteristics may be an attractive approach for future treatment.
authors: Department of Endocrine Oncology, University Medical Centre Utrecht, Utrecht, The Netherlands.
authors: Koen M A
authors: KM
authors: Dreijerink
authors: Jules L
authors: JL
authors: Derks
authors: Ivana
authors: I
authors: Cataldo
authors: Aldo
authors: A
authors: Scarpa
authors: Gerlof D
authors: GD
authors: Valk
authors: Ernst-Jan M
authors: EJ
authors: Speel
mesh_terms: D000818:Animals; D044127:Epigenesis, Genetic / Q000235:genetics*; D006801:Humans; D008175:Lung Neoplasms / Q000235:genetics*; D018358:Neuroendocrine Tumors / Q000235:genetics*; D010190:Pancreatic Neoplasms / Q000235:genetics*