Poremba C, Hero B, Goertz H G, Scheel C, Wai D, Schaefer K L, Christiansen H, Berthold F, Juergens H, Boecker W, Dockhorn-Dworniczak B
Gerhard-Domagk-Institute of Pathology, Westfälische Wilhelms-University, Domagkstrasse 17, 48149 Münster, Germany.
Klin Padiatr. 2001 Jul-Aug;213(4):186-90. doi: 10.1055/s-2001-16850.
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.
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.
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.
神经母细胞瘤（NB）是一组异质性儿童肿瘤，肿瘤进展的可能性范围广泛。由于传统参数不能确保完全准确的预后分组，因此需要新的分子标志物来更精确地评估个体患者的预后。
采用盲法试验分析133例各期NB的端粒酶活性（TA）、存活蛋白表达及MYCN状态。这些数据与其他传统预后指标及疾病转归相关。
TA是所有分期的强大独立预后标志物，能够在NB患者假定的“有利”临床或生物学亚组中区分良好和不良预后。高存活蛋白表达与不良预后相关，但比TA更难解释，因为存活素表达需要准确量化才有预测价值。我们提出了一个扩展的NB进展模型，包括新出现的预后标志物，重点是端粒酶活性。
