Amplification of the MYCN oncogene and deletion of putative tumour suppressor gene in human neuroblastomas

Brain Pathol. 1990 Sep;1(1):41-6. doi: 10.1111/j.1750-3639.1990.tb00637.x.

Abstract

Human neuroblastoma cells often carry non-random chromosomal abnormalities signalling genetic alterations. Quite frequent are 'double minutes' (DMs) and homogeneously staining regions (HSRs), both cytogenetic manifestations of amplified DNA, and chromosome 1p-deletions indicating loss of genetic information. With the identification of amplified MYCN and the demonstration of a consensus deletion spanning the chromosome 1p36.1-2 region it appears now likely that both amplification of a cellular oncogene and loss of a tumour-suppressor gene play an important role in neuroblastoma. Amplification of MYCN is an indicator for poor prognosis, even when classical morphological criteria would suggest a better outcome. Consequently, patients with amplification are subjected to more intensive therapeutic regimens. Amplification of MYCN is a paradigm for the clinical use of an oncogene alteration.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Child
  • Child, Preschool
  • Chromosomes, Human, Pair 1* / ultrastructure
  • Chromosomes, Human, Pair 2 / ultrastructure
  • Gene Amplification*
  • Gene Deletion*
  • Gene Expression Regulation, Neoplastic
  • Genes, Tumor Suppressor*
  • Genes, myc*
  • Humans
  • Incidence
  • Infant
  • Infant, Newborn
  • Neuroblastoma / epidemiology
  • Neuroblastoma / genetics*
  • Peripheral Nervous System Neoplasms / epidemiology
  • Peripheral Nervous System Neoplasms / genetics*
  • Prognosis