Mutation of the multiple endocrine neoplasia type 1 gene in nonfamilial, malignant tumors of the endocrine pancreas

Cancer Res. 1998 Feb 1;58(3):377-9.

Abstract

Endocrine pancreatic tumors are rare neoplasms that occur sporadically or as part of a multiple endocrine neoplasia type 1 (MEN1) syndrome. Germ-line mutations of the MEN1 gene, located at 11q13, have been demonstrated in MEN1 kindreds, and loss of heterozygosity (LOH) on 11q13 together with somatic MEN1 mutations have been detected in 20% of nonfamilial parathyroid tumors. Here, we examine 11 non-MEN1 malignant tumors of the endocrine pancreas, 9 nonfunctioning tumors, and 2 glucagonomas. LOH of at least one informative locus on 11q13 was found in 70% of the tumors. Three tumors displayed somatic mutations of the MEN1 gene together with LOH on 11q13, whereas the corresponding germ-line DNA was normal. These findings support the hypothesis that MEN1 gene mutations contribute to the tumorigenesis of nonfamilial, malignant endocrine pancreatic tumors.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Chromosomes, Human, Pair 11 / genetics
  • DNA, Neoplasm / genetics
  • Female
  • Glucagonoma / genetics*
  • Humans
  • Loss of Heterozygosity
  • Male
  • Middle Aged
  • Neoplasm Proteins / deficiency
  • Neoplasm Proteins / genetics*
  • Pancreatic Neoplasms / genetics*
  • Polymerase Chain Reaction
  • Proto-Oncogene Proteins*

Substances

  • DNA, Neoplasm
  • MEN1 protein, human
  • Neoplasm Proteins
  • Proto-Oncogene Proteins