Wieczorek Aleksandra, Balwierz Walentyna
Klinika Onkologii i Hematologii Dzieciecej, Polsko-Amerykański Instytut Pediatrii, UJ CM, Kraków.
Przegl Lek. 2010;67(6):393-8.
Neuroblastoma (NBL) accounts for 6-10% of neoplastic diseases of childhood. The clinical course of NBL is very variable and depends on the presence of prognostic factors. The aim of the study is retrospective evaluation of occurrence of known prognostic factors and disease markers in patients treated in 1991-2005 in our institution. Sixty of 100 treated patients were included to the study, in whom tumor embedded in paraffin was available. In all patients, who had no genetic evaluation at diagnosis, especially MYCN amplification, FISH study was performed. In analyzed group, 29 patients died, 23 of them from NBL. Disease progression (n = 12) or relapse (n = 19) was observed in 31 patients. In the whole analyzed group, age had statistically significant influence on deaths caused by NBL (p = 0.01) and therapy failures (p = 0.00008). The statistically significant increase of NBL death incidence (p = 0.00001) and therapy failures (p = 0.00004) was found in stage 4 patients in comparison with other stages. Presence of MYCN amplification statistically significant decreases overall survival (OS) (p = 0.01) and disease free survival (DFS) (p = 0.047) for the whole analyzed group. However, presence of MYCN amplification had no statistically significant influence on OS and DFS in patients over 1 year of age. Multiple Cox analysis showed independent statistically significant influence of stage and MYCN amplification on prognosis. Employment of new treatment modalities, with treatment intensity adjusted to the risk group, but also with the specificity adapted to tumor characteristics (cytogenetic and molecular) as well as further development of supportive therapy improvement may lead to improvement of treatment results, especially in high risk group and to reduction of therapy complications and improvement of quality of life in all children with this tumor.
神经母细胞瘤（NBL）占儿童肿瘤性疾病的6 - 10%。NBL的临床病程变化很大，取决于预后因素的存在情况。本研究的目的是对1991年至2005年在我们机构接受治疗的患者中已知预后因素和疾病标志物的发生情况进行回顾性评估。100例接受治疗的患者中有60例被纳入研究，这些患者有石蜡包埋的肿瘤组织。在所有诊断时未进行基因评估，尤其是未评估MYCN扩增的患者中，均进行了荧光原位杂交（FISH）研究。在分析组中，29例患者死亡，其中23例死于NBL。31例患者观察到疾病进展（n = 12）或复发（n = 19）。在整个分析组中，年龄对NBL导致的死亡（p = 0.01）和治疗失败（p = 0.00008）有统计学显著影响。与其他分期相比，4期患者中NBL死亡发生率（p = 0.00001）和治疗失败率（p = 0.00004）有统计学显著增加。对于整个分析组，MYCN扩增的存在使总生存期（OS）（p = 0.01）和无病生存期（DFS）（p = 0.047）有统计学显著降低。然而，MYCN扩增的存在对1岁以上患者的OS和DFS没有统计学显著影响。多因素Cox分析显示分期和MYCN扩增对预后有独立的统计学显著影响。采用新的治疗模式，将治疗强度调整至风险组，同时使其特异性适应肿瘤特征（细胞遗传学和分子特征），以及进一步改进支持治疗，可能会改善治疗效果，尤其是在高危组中，并减少治疗并发症，提高所有患此肿瘤儿童的生活质量。
