Amplification of the MYCN oncogene and deletion of putative tumour suppressor gene in human neuroblastomas.
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.
Schwab
M
M
Institute for Experimental Pathology, German Cancer Research Center, Heidelberg.
D002648:Child; D002675:Child, Preschool; D002878:Chromosomes, Human, Pair 1 / Q000648:ultrastructure; D002889:Chromosomes, Human, Pair 2 / Q000648:ultrastructure; D005784:Gene Amplification; D017353:Gene Deletion; D015972:Gene Expression Regulation, Neoplastic; D016147:Genes, Tumor Suppressor; D016259:Genes, myc; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D009447:Neuroblastoma / Q000453:epidemiology / Q000235:genetics*; D010524:Peripheral Nervous System Neoplasms / Q000453:epidemiology / Q000235:genetics*; D011379:Prognosis