92例儿童神经母细胞瘤的临床特征及预后分析

发布时间：2018-03-30 04:22

本文选题：神经母细胞瘤　切入点：儿童　出处：《上海交通大学》2014年硕士论文

【摘要】：目的探讨儿童神经母细胞瘤（Neuroblastoma，NB）的临床特征及预后影响因素。方法回顾性分析2007年1月至2012年12月我院收治且随访资料完整的92例NB患儿临床资料，比较不同因素对预后的影响。结果92例患儿中，男女比例为1.5:1，中位诊断年龄为32.3个月（2.0-159.8个月）,中位随访时间为32个月（12-80个月）。5年总体生存率（5y-OS）为65.9%±5.4%，5年无事件生存率（5y-EFS）为56.2%±5.7%。92例NB中29例出现复发、进展，中位复发进展时间为确诊后11.0个月（1.1-27.5个月），复发、进展后3y-OS为23.5%±8.8%，诊断后18个月内复发的患儿较18个月后复发的患儿预后差（7.3%±6.8%vs80.0%±17.9%，P=0.006）。本组共有28例（30.4%）患儿死亡，死亡首要原因是肿瘤进展所致（20/28,71.4%），，其次为化疗相关的严重感染（6/28,21.4%）。单因素分析显示INSS分期（P=0.001）、危险度分层（P=0.001）、LDH＞500U/L （P=0.033）、骨骼转移（P=0.004）、N-myc基因扩增（P=0.016）是影响总体生存率的重要因素。多因素分析显示INSS分期（P=0.013）、N-myc基因扩增（P=0.022）为独立的预后影响因素。结论INSS分期和N-myc基因的扩增状态是影响NB患者预后的独立因素。应重视强烈化疗后并发严重感染导致的死亡事件。诊断后18个月内复发的患儿预后较差，但I和II期或仅原发部位提示复发的患儿，在首次复发后予以积极治疗仍可获得长期生存。联合治疗包括化疗、手术、放疗和自体干细胞移植可改善高危与极高危NB患儿的预后。
[Abstract]:Objective to investigate the clinical features and prognostic factors of neuroblastomaena NB in children with neuroblastomas. Methods the clinical data of 92 cases of NB patients with complete follow-up data from January 2007 to December 2012 were analyzed retrospectively and the effects of different factors on prognosis were compared. Results in 92 cases, the ratio of male to female was 1.5: 1, the median diagnostic age was 32.3 months (2.0-159.8 months), the median follow-up time was 32 months (12-80 months), the overall 5-year survival rate (5y-OS) was 65.9% 卤5.4%, the 5-year event-free survival rate (5y-EFS) was 56.2% 卤5.7.92 cases of NB recurred and progressed. The median time of recurrence and progression was 1.1-27.5 months after diagnosis, and the 3y-OS after progression was 23.5% 卤8.8%. The prognosis of children with recurrence within 18 months after diagnosis was 7.3% 卤6.8vs80.0% 卤17.9% 卤17.9P0.006. There were 28 cases of death in this group. The leading cause of death was 20 / 28 / 71.4%, followed by chemotherapy-related severe infection (6 / 28 / 21. 4). Univariate analysis showed that INSS staging was 0. 001%, 500U/L was higher than P0. 033, bone metastasis P0. 004 N-myc gene amplification (P0. 016) was an important factor affecting overall survival rate. Factor analysis showed that INSS staging was an independent prognostic factor with the amplification of N-myc gene (P0. 022). Conclusion the stage of INSS and the amplification status of N-myc gene are independent factors influencing the prognosis of NB patients. We should pay attention to the death events caused by severe infection after intensive chemotherapy. The prognosis of children with recurrence within 18 months after diagnosis is poor. However, children with stage I and II or only primary sites who indicate recurrence may still be able to survive long term after active treatment after the first recurrence. Combination therapy includes chemotherapy, surgery, Radiotherapy and autologous stem cell transplantation can improve the prognosis of children with high and very high risk NB.
【学位授予单位】：上海交通大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R739.4

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