PMID- 9287968 OWN - NLM STAT- MEDLINE DCOM- 19970930 LR - 20190707 IS - 0016-5085 (Print) IS - 0016-5085 (Linking) VI - 113 IP - 3 DP - 1997 Sep TI - The multiple endocrine neoplasia type I gene locus is involved in the pathogenesis of type II gastric carcinoids. PG - 773-81 AB - BACKGROUND & AIMS: Both gastrin and genetic factors were suggested to underlie the pathogenesis of multiple gastric enterochromaffin-like (ECL) cell carcinoids. To assess the role of genetic alterations in carcinoid tumorigenesis, loss of heterozygosity (LOH) at the locus of the multiple endocrine neoplasia type 1 (MEN-1) gene was studied in gastric carcinoids of patients with MEN-1 and chronic atrophic type A gastritis (A-CAG), as well as in sporadically arising intestinal carcinoids. METHODS: DNA extracted from archival tissue sections of 35 carcinoid tumors was assessed for LOH with eight polymorphic markers on chromosome 11q13. A combined tumor and family study was performed in 1 patient with MEN-1-Zollinger-Ellison syndrome (ZES). RESULTS: LOH at 11q13 loci was detected in 15 of 20 (75%) MEN-1-ZES carcinoids, and each ECL-cell carcinoid with LOH showed deletion of the wild-type allele. Only 1 of 6 A-CAG carcinoids displayed LOH at the MEN-1 gene locus, and none of the 9 intestinal and rectal carcinoids showed 11q13 LOH. CONCLUSIONS: Gastric ECL-cell carcinoid is an independent tumor type of MEN-1 that shares a common developmental mechanism (via inactivation of the MEN-1 gene) with enteropancreatic and parathyroid MEN-1 tumors. Further analysis of sporadic and A-CAG carcinoids is needed to elucidate genetic factors involved in their tumorigenesis. FAU - Debelenko, L V AU - Debelenko LV AD - Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland, USA. FAU - Emmert-Buck, M R AU - Emmert-Buck MR FAU - Zhuang, Z AU - Zhuang Z FAU - Epshteyn, E AU - Epshteyn E FAU - Moskaluk, C A AU - Moskaluk CA FAU - Jensen, R T AU - Jensen RT FAU - Liotta, L A AU - Liotta LA FAU - Lubensky, I A AU - Lubensky IA LA - eng PT - Journal Article PL - United States TA - Gastroenterology JT - Gastroenterology JID - 0374630 RN - 0 (DNA, Neoplasm) RN - 0 (Genetic Markers) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Carcinoid Tumor/*genetics/pathology MH - *Chromosome Mapping MH - Chromosomes, Human, Pair 11/genetics MH - DNA, Neoplasm/genetics MH - Female MH - Gastritis/genetics MH - Gene Deletion MH - Genetic Markers MH - Humans MH - Intestinal Neoplasms/genetics MH - Male MH - Middle Aged MH - Multiple Endocrine Neoplasia Type 1/*genetics MH - Stomach Neoplasms/*genetics/pathology MH - Zollinger-Ellison Syndrome/genetics/pathology EDAT- 1997/09/01 00:00 MHDA- 1997/09/01 00:01 CRDT- 1997/09/01 00:00 PHST- 1997/09/01 00:00 [pubmed] PHST- 1997/09/01 00:01 [medline] PHST- 1997/09/01 00:00 [entrez] AID - S0016508597004149 [pii] AID - 10.1016/s0016-5085(97)70171-9 [doi] PST - ppublish SO - Gastroenterology. 1997 Sep;113(3):773-81. doi: 10.1016/s0016-5085(97)70171-9.