Balwierz Walentyna
Klinika Onkologii i Hematologii Dzieciecej Polsko-Amerykańskiego, Instytutu Pediatrii, Collegium Medicum, Uniwersytetu Jagiellońskiego w Krakowie.
Przegl Lek. 2004;61 Suppl 2:3-8.
Neuroblastoma is one of the most common (7-10%) childhood cancers and the most frequent malignancy in neonates and infants. The disease course depends not only on the child's age and clinical stage but also on other factors, especially cytogenetic and molecular abnormalities. The currently employed treatment modalities should cure at least 60% of all neuroblastoma patients. But, in a very high risk group (age >1 year, stages 2 and 3 with N-MYC amplification, all patients with stage 4), which constitutes more than half of patients, results of very intensive treatment remain still unsatisfactory. Fewer than one-third of children over one year of age with stage 4 neuroblastoma are expected to survive. The success of treatment depends not only on treatment, which intensity is tailored to the risk of individual patients, but also on detailed and precise diagnosis and therapy monitoring. Children with neuroblastoma, regardless of the presenting signs and age, should undergo comprehensive evaluation, including genetics and molecular studies. Majority of infants with neuroblastoma have a good prognosis, but choice of therapy in these patients depends on disease stage, critical organ function (Philadelphia score), surgical treatment possibilities and on the presence of the N-MYC amplification. Further improvement of therapeutic strategy, especially for advanced neuroblastoma, calls for associating individual centers in large groups, which would cooperate in completing research projects, which currently are being implemented in Europe. Novel treatment modalities should improve the treatment results in the high risk group, as well as decrease the late side-effects number in all children treated for neuroblastoma.
神经母细胞瘤是最常见的儿童癌症之一(占7%-10%),也是新生儿和婴儿中最常见的恶性肿瘤。疾病进程不仅取决于患儿的年龄和临床分期,还取决于其他因素,尤其是细胞遗传学和分子异常情况。目前采用的治疗方式应能治愈至少60%的神经母细胞瘤患者。但是,在占患者总数一半以上的极高危组(年龄>1岁,2期和3期且伴有N-MYC扩增,所有4期患者)中,强化治疗的效果仍然不尽人意。预计1岁以上的4期神经母细胞瘤患儿中,存活者不到三分之一。治疗的成功不仅取决于根据个体患者风险调整强度的治疗,还取决于详细精确的诊断和治疗监测。患有神经母细胞瘤的儿童,无论出现何种体征和年龄,都应接受包括遗传学和分子研究在内的全面评估。大多数神经母细胞瘤婴儿预后良好,但这些患者的治疗选择取决于疾病分期、关键器官功能(费城评分)、手术治疗可能性以及N-MYC扩增情况。治疗策略的进一步改进,尤其是针对晚期神经母细胞瘤,需要各大团体中的各个中心联合起来,共同合作完成目前正在欧洲开展的研究项目。新的治疗方式应能改善高危组的治疗效果,并减少所有接受神经母细胞瘤治疗儿童的晚期副作用数量。