The multiple endocrine neoplasia type I gene locus is involved in the pathogenesis of type II gastric carcinoids

Gastroenterology. 1997 Sep;113(3):773-81. doi: 10.1016/s0016-5085(97)70171-9.


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.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Carcinoid Tumor / genetics*
  • Carcinoid Tumor / pathology
  • Chromosome Mapping*
  • Chromosomes, Human, Pair 11 / genetics
  • DNA, Neoplasm / genetics
  • Female
  • Gastritis / genetics
  • Gene Deletion
  • Genetic Markers
  • Humans
  • Intestinal Neoplasms / genetics
  • Male
  • Middle Aged
  • Multiple Endocrine Neoplasia Type 1 / genetics*
  • Stomach Neoplasms / genetics*
  • Stomach Neoplasms / pathology
  • Zollinger-Ellison Syndrome / genetics
  • Zollinger-Ellison Syndrome / pathology


  • DNA, Neoplasm
  • Genetic Markers