title: Number of genomic imbalances correlates with the overall survival for adrenocortical cancer in childhood.
abstract: BACKGROUND
  Adrenocortical tumours (ACT) in children are rare and, if malignant, often associated with poor prognosis. Relevant cytogenetic factors for prognosis are hardly available.
  
  
  METHODS
  We analysed 14 adrenocortical cancers (ACC) of children by comparative genomic hybridisation (CGH).
  
  
  RESULTS
  The total number of genomic imbalances ranged from 1 to 17 in individual tumour samples. The most common imbalances were +1q (57%), +12p (50%), +12q (50%), +1p (43%), +7q (42%), +9q (42%), +15q (42%), and -4q (57%), -11q (57%), -4p (42%), and -16q (42%). The median number of genomic changes was 5.5 (n = 8) in pT1-pT2 and 15.5 (n = 6) in pT3-pT4 tumours. The median number was 4 in the eight patients, who remain in remission more than 51 months and 15.5 in the six patients, who have died from the disease within 44 months. Moreover, all seven patients with less than 10 individual imbalances were in remission (median follow-up 72 months), while all but one patient with 10 and more individual imbalances (n = 7) have died from the disease (median survival time 30 months). Comparison of the data from children and adults revealed characteristic differences. Gain of 1p and loss of 4p, 4q and 16q are frequent in childhood and rare in adults. Inversely, loss of 1p is rare in childhood but frequent in adult ACT.
  
  
  CONCLUSIONS
  The number of CGH imbalances appeared to have a predictive value for overall survival in paediatric ACC.
authors.affiliation: Institute for Human Genetics and Anthropology, Clinical University Hospital, Jena, Germany. ivan@clondiag.com
authors.forename: Ivan F
authors.initials: IF
authors.lastname: Loncarevic
authors.forename: Antje
authors.initials: A
authors.lastname: Hering
authors.forename: Nicole
authors.initials: N
authors.lastname: Posorski
authors.forename: Tobias
authors.initials: T
authors.lastname: Linden
authors.forename: Heike
authors.initials: H
authors.lastname: Hoyer
authors.forename: Peter
authors.initials: P
authors.lastname: Bucsky
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000235:genetics* / Q000401:mortality*; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations; D020732:Cytogenetic Analysis; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009693:Nucleic Acid Hybridization; D011379:Prognosis; D015996:Survival Rate