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