PMID- 18341492 OWN - NLM STAT- MEDLINE DCOM- 20080606 LR - 20091119 IS - 0002-9270 (Print) IS - 0002-9270 (Linking) VI - 103 IP - 3 DP - 2008 Mar TI - Molecular genetics of gastroenteropancreatic neuroendocrine tumors. PG - 729-32 LID - 10.1111/j.1572-0241.2007.01777.x [doi] AB - Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are usually sporadic; however, familial (inherited) syndromes, such as the multiple endocrine neoplasia 1 (MEN-1) syndrome, von Hippel-Lindau (VHL) syndrome, neurofibromatosis (NF-1), as well as tuberous sclerosis, may be associated with proximal intestinal and pancreatic NETs. For example, 25% of gastrinoma patients have MEN-1 syndrome. Over the last two decades, the genetic basis of tumorigenesis for these familial syndromes has been clearly identified, providing clinicians with useful screening tools for affected families. Also, over the last few years, advanced molecular genetic techniques, such as comparative genomic hybridization (CGH) and loss of heterozygosity (LOH) analyses, have detected some differences in genomic aberrations among various types of NETs. Whether these chromosomic alterations have implications in the treatment of patients and the outcome of the disease is still unclear. FAU - Toumpanakis, Christos G AU - Toumpanakis CG AD - Neuroendocrine Tumour Unit, Centre for Gastroenterology, Royal Free Hospital, London, United Kingdom. FAU - Caplin, Martyn E AU - Caplin ME LA - eng PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Review PL - United States TA - Am J Gastroenterol JT - The American journal of gastroenterology JID - 0421030 SB - IM MH - Adenoma, Islet Cell/diagnosis/genetics MH - Carcinoid Tumor/diagnosis/genetics MH - Gastrointestinal Neoplasms/diagnosis/*genetics MH - Genetic Testing MH - Humans MH - Neoplastic Syndromes, Hereditary/diagnosis/genetics MH - Neuroendocrine Tumors/diagnosis/*genetics MH - Pancreatic Neoplasms/diagnosis/*genetics RF - 26 EDAT- 2008/03/18 09:00 MHDA- 2008/06/07 09:00 CRDT- 2008/03/18 09:00 PHST- 2008/03/18 09:00 [pubmed] PHST- 2008/06/07 09:00 [medline] PHST- 2008/03/18 09:00 [entrez] AID - AJG1777 [pii] AID - 10.1111/j.1572-0241.2007.01777.x [doi] PST - ppublish SO - Am J Gastroenterol. 2008 Mar;103(3):729-32. doi: 10.1111/j.1572-0241.2007.01777.x.