Traditional and emerging molecular markers in neuroblastoma prognosis: the good, the bad and the ugly.
BACKGROUND
Neuroblastomas (NB) are a heterogeneous group of childhood tumours with a wide range of likelihood for tumour progression. As traditional parameters do not ensure completely accurate prognostic grouping, new molecular markers are needed for assessing the individual patient's prognosis more precisely.


METHODS
133 NB of all stages were analysed in blind-trial fashion for telomerase activity (TA), expression of surviving, and MYCN status. These data were correlated with other traditional prognostic indicators and disease outcome.


CONCLUSIONS
TA is a powerful independent prognostic marker for all stages and is capable of differentiating between good and poor outcome in putative "favourable" clinical or biological subgroups of NB patients. High surviving expression is associated with an adverse outcome, but is more difficult to interprete than TA because survivin expression needs to be accurately quantified to be of predictive value. We propose an extended progression model for NB including emerging prognostic markers, with emphasis on telomerase activity.
Poremba
C
C
Gerhard-Domagk-Institute of Pathology, Westfälische Wilhelms-University, Domagkstrasse 17, 48149 Münster, Germany. poremba@uni-muenster.de
Hero
B
B
Goertz
H G
HG
Scheel
C
C
Wai
D
D
Schaefer
K L
KL
Christiansen
H
H
Berthold
F
F
Juergens
H
H
Boecker
W
W
Dockhorn-Dworniczak
B
B
D014408:Biomarkers, Tumor / Q000235:genetics*; D015139:Blotting, Southern; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy; D002675:Child, Preschool; D002868:Chromosomal Proteins, Non-Histone / Q000235:genetics*; D018450:Disease Progression; D005260:Female; D016231:Fluorescence Polarization Immunoassay; D005500:Follow-Up Studies; D015972:Gene Expression Regulation, Neoplastic; D016259:Genes, myc / Q000235:genetics*; D006801:Humans; D066298:In Vitro Techniques; D007223:Infant; D051034:Inhibitor of Apoptosis Proteins; D008297:Male; D008869:Microtubule-Associated Proteins; D008954:Models, Biological; D009363:Neoplasm Proteins; D009447:Neuroblastoma / Q000175:diagnosis* / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D016037:Single-Blind Method; D016019:Survival Analysis; D000077022:Survivin; D019098:Telomerase / Q000235:genetics*; D016896:Treatment Outcome
C107489:BIRC5 protein, human; D014408:Biomarkers, Tumor; D002868:Chromosomal Proteins, Non-Histone; D051034:Inhibitor of Apoptosis Proteins; D008869:Microtubule-Associated Proteins; D009363:Neoplasm Proteins; D000077022:Survivin; D019098:Telomerase