5岁以上神经母细胞瘤患者的临床特点及疗效分析

Clinical features and outcomes of neuroblastoma patients aged above 5 years

摘要:

目的 总结5岁以上神经母细胞瘤（NB）患儿的临床特点及疗效，为改善其预后提供理论基础。方法 回顾性分析54例初治NB患儿临床资料，进行临床特点、疗效总结以及Kaplan-Meier生存分析。结果 54例患儿中男36例、女18例，均为3、4期。以腹膜后占位最多见（76%，41/54），其次为纵隔占位（18%，10/54）、椎管内占位（4%，2/54）和盆腔占位（2%，1/54）。截止至随访日期，54例患儿中存活30例（56%）：无病生存23例（77%，其中9例为复发再度化疗后完全缓解）、肿瘤部分缓解6例（20%，均为复发再度化疗患儿）、进展1例（3%，为复发再度化疗后进展）；死亡24例（44%），其中22例为复发再度化疗后死亡、2例为初治期间多脏器衰竭死亡；治疗、随访期间共38例复发。Kaplan-Meier生存分析提示：54例患儿平均生存时间53.8个月；3期患儿总生存率（OS）为80%，高于4期患儿（53%），差异具有统计学意义（P < 0.01）；复发患儿平均生存时间（51.68个月）低于无复发病例（62.57个月），差异有统计学意义（P < 0.01）。结论 年长儿NB临床分期多属晚期，但规律治疗仍可提高疗效，应增加患儿信心，坚持规范治疗。

Abstract:

Objective To investigate the clinical features and outcomes of neuroblastoma (NB) children aged above 5 years, and to provide a theoretical basis for improving prognosis. Methods A retrospective analysis was performed for the clinical data of 54 previously untreated NB children, and their clinical features and outcome were analyzed. The Kaplan-Meier method was used for survival analysis. Results Among the 54 children, there were 36 boys and 18 girls, and all of them had stage 3 or 4 NB. Of all the children, 41 (41/54, 76%) had retroperitoneal space-occupying lesions, 10 (10/54, 18%) had mediastinal space-occupying lesions, 2 (2/54, 4%) had intraspinal space-occupying lesions, and 1 (1/54, 2%) had pelvic space-occupying lesions. At the end of the follow-up, 30 children (30/54, 56%) survived, among whom 23 (77%) achieved disease-free survival (9 achieved complete remission after chemotherapy for recurrence), 6 (20%) achieved partial remission of tumor (all of them received chemotherapy again due to recurrence), and 1 (3%) experienced progression (with progression after chemotherapy again due to recurrence); 24 children (44%) died, among whom 22 died after chemotherapy again due to recurrence and 2 died of multiple organ failure during the first treatment. According to the Kaplan-Meier survival analysis, the mean survival time was 53.8 months, and the children with stage 3 NB had a significantly higher overall survival rate than those with stage 4 NB (80% vs 53%;P < 0.01). The children with recurrence had a significantly lower mean survival time than those without recurrence (51.68 months vs 62.57 months;P < 0.01). Conclusions Older children often have late-stage NB, but standard treatment can improve their outcomes.