title: Gain of chromosome arm 17q and adverse outcome in patients with neuroblastoma.
abstract: BACKGROUND
  Gain of genetic material from chromosome arm 17q (gain of segment 17q21-qter) is the most frequent cytogenetic abnormality of neuroblastoma cells. This gain has been associated with advanced disease, patients who are > or =1 year old, deletion of chromosome arm 1p, and amplification of the N-myc oncogene, all of which predict an adverse outcome. We investigated these associations and evaluated the prognostic importance of the status of chromosome 17.
  
  
  METHODS
  We compiled molecular cytogenetic analyses of chromosome 17 in primary neuroblastomas in 313 patients at six European centers. Clinical and survival information were collected, along with data on 1p, N-myc, and ploidy.
  
  
  RESULTS
  Unbalanced gain of segment 17q21-qter was found in 53.7 percent of the tumors, whereas the chromosome was normal in 46.3 percent. The gain of 17q was characteristic of advanced tumors and of tumors in children > or =1 year of age and was strongly associated with the deletion of 1p and amplification of N-myc. No tumor showed amplification of N-myc in the absence of either deletion of 1p or gain of 17q. Gain of 17q was a significant predictive factor for adverse outcome in univariate analysis. Among the patients with this abnormality, overall survival at five years was 30.6 percent (95 percent confidence interval, 21 to 40 percent), as compared with 86.0 percent (95 percent confidence interval, 78 to 91 percent) among those with normal 17q status. in multivariate analysis, gain of 17q was the most powerful prognostic factor, followed by the presence of stage 4 disease and deletion of 1p (hazard ratios, 3.4, 2.3, and 1.9, respectively).
  
  
  CONCLUSIONS
  Gain of chromosome segment 17q21-qter is an important prognostic factor in children with neuroblastoma.
authors.affiliation: Department of Human Genetics, University of Newcastle upon Tyne, United Kingdom. nicholas.bown@ncl.ac.uk
authors.forename: N
authors.initials: N
authors.lastname: Bown
authors.forename: S
authors.initials: S
authors.lastname: Cotterill
authors.forename: M
authors.initials: M
authors.lastname: Lastowska
authors.forename: S
authors.initials: S
authors.lastname: O'Neill
authors.forename: A D
authors.initials: AD
authors.lastname: Pearson
authors.forename: D
authors.initials: D
authors.lastname: Plantaz
authors.forename: M
authors.initials: M
authors.lastname: Meddeb
authors.forename: G
authors.initials: G
authors.lastname: Danglot
authors.forename: C
authors.initials: C
authors.lastname: Brinkschmidt
authors.forename: H
authors.initials: H
authors.lastname: Christiansen
authors.forename: G
authors.initials: G
authors.lastname: Laureys
authors.forename: F
authors.initials: F
authors.lastname: Speleman
authors.forename: J
authors.initials: J
authors.lastname: Nicholson
authors.forename: A
authors.initials: A
authors.lastname: Bernheim
authors.forename: D R
authors.initials: DR
authors.lastname: Betts
authors.forename: J
authors.initials: J
authors.lastname: Vandesompele
authors.forename: N
authors.initials: N
authors.lastname: Van Roy
mesh_terms: D000704:Analysis of Variance; D002675:Child, Preschool; D002872:Chromosome Deletion; D002878:Chromosomes, Human, Pair 1 / Q000235:genetics; D002886:Chromosomes, Human, Pair 17; D005784:Gene Amplification; D016259:Genes, myc / Q000235:genetics; D006801:Humans; D007223:Infant; D015999:Multivariate Analysis; D009367:Neoplasm Staging; D009447:Neuroblastoma / Q000235:genetics* / Q000401:mortality / Q000473:pathology; D011379:Prognosis; D012044:Regression Analysis; D012307:Risk Factors; D015996:Survival Rate; D014178:Translocation, Genetic