Neuroblastoma tumour genetics: clinical and biological aspects.
Neuroblastoma tumour cells show complex combinations of acquired genetic aberrations, including ploidy changes, deletions of chromosome arms 1p and 11q, amplification of the MYCN oncogene, and-most frequently-gains of chromosome arm 17q. Despite intensive investigation, the fundamental role of these features in neuroblastoma initiation and progression remains to be understood. Nonetheless, great progress has been made in relating tumour genetic abnormalities to tumour behaviour and to clinical outcome; indeed, neuroblastoma provides a paradigm for the clinical importance of tumour genetic abnormalities. Knowledge of MYCN status is increasingly being used in treatment decisions for individual children, and the clinical value of 1p and 17q data as adjuncts or refinements in risk stratification is under active investigation. Reliable detection of these molecular cytogenetic features should be regarded as mandatory for all new cases at presentation.
Bown
N
N
School of Biochemistry and Genetics, University of Newcastle upon Tyne/Northern Genetics Service, Royal Victoria Infirmary, 19/20 Claremont Place, Newcastle upon Tyne NE2 4AA, UK. Nick.Brown@ncl.ac.uk
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002868:Chromosomal Proteins, Non-Histone / Q000235:genetics; D002869:Chromosome Aberrations; D002886:Chromosomes, Human, Pair 17; D029721:Drosophila Proteins; D005784:Gene Amplification; D015870:Gene Expression; D019450:Genes, MDR; D006801:Humans; D018960:Hyaluronan Receptors / Q000235:genetics; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D051034:Inhibitor of Apoptosis Proteins; D019476:Insect Proteins / Q000235:genetics; D019656:Loss of Heterozygosity; D008869:Microtubule-Associated Proteins; D020559:Monomeric GTP-Binding Proteins / Q000235:genetics; D054778:NM23 Nucleoside Diphosphate Kinases; D009363:Neoplasm Proteins; D009447:Neuroblastoma / Q000235:genetics*; D009701:Nucleoside-Diphosphate Kinase; D011003:Ploidies; D016271:Proto-Oncogene Proteins c-myc / Q000235:genetics; D000077022:Survivin; D019098:Telomerase / Q000235:genetics; D014157:Transcription Factors / Q000235:genetics
C107489:BIRC5 protein, human; D002868:Chromosomal Proteins, Non-Histone; D029721:Drosophila Proteins; D018960:Hyaluronan Receptors; D051034:Inhibitor of Apoptosis Proteins; D019476:Insect Proteins; D008869:Microtubule-Associated Proteins; D054778:NM23 Nucleoside Diphosphate Kinases; D009363:Neoplasm Proteins; D016271:Proto-Oncogene Proteins c-myc; D000077022:Survivin; D014157:Transcription Factors; C096356:trk protein, Drosophila; D009701:Nucleoside-Diphosphate Kinase; D019098:Telomerase; D020559:Monomeric GTP-Binding Proteins