PMID- 17382262 OWN - NLM STAT- MEDLINE DCOM- 20070613 LR - 20071115 IS - 1521-690X (Print) IS - 1521-690X (Linking) VI - 21 IP - 1 DP - 2007 Mar TI - Molecular genetics of neuroendocrine tumors. PG - 1-14 AB - Neuroendocrine tumors can develop either sporadically or in association with familial syndromes such as multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2 (MEN2) or von Hippel-Lindau (VHL). A variety of genetic approaches has been utilized to dissect the underlying molecular pathogenesis of these distinctive tumors, including genome-wide screens such as comparative genomic hybridization, loss of heterozygosity and DNA microarray analysis as well as targeted investigations into specific tumor suppressor gene and oncogene candidates. The identification of the MEN1 tumor suppressor gene that underlies the MEN1 syndrome has provided important new insights into tumor pathogenesis. In addition, a number of independent approaches has converged on a pivotal role for regulators of the cell cycle. However, our understanding of the molecular biology of these tumors remains far from complete. In this review we highlight some of the key approaches, findings and implications of these genetic studies. FAU - Duerr, Eva-Maria AU - Duerr EM AD - Gastrointestinal Unit, Department of Medicine, Massachusetts General Hospital, Boston, MA 02114, USA. FAU - Chung, Daniel C AU - Chung DC LA - eng PT - Journal Article PT - Review PL - Netherlands TA - Best Pract Res Clin Endocrinol Metab JT - Best practice & research. Clinical endocrinology & metabolism JID - 101120682 SB - IM MH - Animals MH - Cell Cycle/genetics MH - Chromosome Mapping MH - Chromosomes, Human MH - Gene Expression Profiling/methods MH - Humans MH - Loss of Heterozygosity MH - Models, Biological MH - Multiple Endocrine Neoplasia Type 1/genetics MH - Multiple Endocrine Neoplasia Type 2a/genetics MH - Neuroendocrine Tumors/*genetics MH - Nucleic Acid Hybridization/methods MH - Syndrome MH - von Hippel-Lindau Disease/genetics RF - 88 EDAT- 2007/03/27 09:00 MHDA- 2007/06/15 09:00 CRDT- 2007/03/27 09:00 PHST- 2007/03/27 09:00 [pubmed] PHST- 2007/06/15 09:00 [medline] PHST- 2007/03/27 09:00 [entrez] AID - S1521-690X(06)00110-2 [pii] AID - 10.1016/j.beem.2006.12.001 [doi] PST - ppublish SO - Best Pract Res Clin Endocrinol Metab. 2007 Mar;21(1):1-14. doi: 10.1016/j.beem.2006.12.001.