title: Gastroenteropancreatic endocrine tumors.
abstract: Gastroenteropancreatic endocrine tumors (GEP-NETs) are relatively uncommon; comprising approximately 0.5% of all human cancers. Although they often exhibit relatively indolent clinical courses, GEP-NETs have the potential for lethal progression. Due to their scarcity and various technical challenges, GEP-NETs have been understudied. As a consequence, we have few diagnostic, prognostic and predictive biomarkers for these tumors. Early detection and surgical removal is currently the only reliable curative treatment for GEP-NET patients; many of whom, unfortunately, present with advanced disease. Here, we review the genetics and epigenetics of GEP-NETs. The last few years have witnessed unprecedented technological advances in these fields, and their application to GEP-NETS has already led to important new information on the molecular abnormalities underlying them. As outlined here, we expect that "omics" studies will provide us with new diagnostic and prognostic biomarkers, inform the development of improved pre-clinical models, and identify novel therapeutic targets for GEP-NET patients.
authors.affiliation: The Johns Hopkins University School of Medicine, Department of Pathology, Bond Street Research Annex Bldg., Room B300, 411 North Caroline Street, Baltimore, MD 21231, United States. Electronic address: ameeker1@jhmi.edu.
authors.forename: Alan
authors.initials: A
authors.lastname: Meeker
authors.affiliation: The Johns Hopkins University School of Medicine, Department of Pathology, Bond Street Research Annex Bldg., Room B300, 411 North Caroline Street, Baltimore, MD 21231, United States.
authors.forename: Christopher
authors.initials: C
authors.lastname: Heaphy
mesh_terms: D000818:Animals; D044127:Epigenesis, Genetic; D005770:Gastrointestinal Neoplasms / Q000235:genetics / Q000473:pathology*; D023281:Genomics; D006801:Humans; D018358:Neuroendocrine Tumors / Q000235:genetics / Q000473:pathology*; D010190:Pancreatic Neoplasms / Q000235:genetics / Q000473:pathology*; D015398:Signal Transduction / Q000235:genetics
keywords: ATRX; Carcinoid; DAXX; Epigenomics; Gastroenteropancreatic Neuroendocrine Tumor; Genomics