靳燕, 袁晓军, 赵强, 李府, 李建新, 江莲, 卢俊, 戴云鹏, 赵平, 詹江华, 李杰, 曹嫣娜, 李璋琳, 杨嘉兴, 李忠元, 王道威, 龚宝成, 闫杰. 神经母细胞瘤CCCG-NB-2015共识多中心应用总结[J]. 中国肿瘤临床, 2023, 50(9): 433-442. DOI: 10.12354/j.issn.1000-8179.2023.20221693
引用本文: 靳燕, 袁晓军, 赵强, 李府, 李建新, 江莲, 卢俊, 戴云鹏, 赵平, 詹江华, 李杰, 曹嫣娜, 李璋琳, 杨嘉兴, 李忠元, 王道威, 龚宝成, 闫杰. 神经母细胞瘤CCCG-NB-2015共识多中心应用总结[J]. 中国肿瘤临床, 2023, 50(9): 433-442. DOI: 10.12354/j.issn.1000-8179.2023.20221693
shu
Yan Jin, Xiaojun Yuan, Qiang Zhao, Fu Li, Jianxin Li, Lian Jiang, Jun Lu, Yunpeng Dai, Ping Zhao, Jianghua Zhan, Jie Li, Yanna Cao, Zhanglin Li, Jiaxing Yang, Zhongyuan Li, Daowei Wang, Baocheng Gong, Jie Yan. Summary of the multicenter application of the CCCG-NB-2015 consensus in neuroblastoma[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2023, 50(9): 433-442. DOI: 10.12354/j.issn.1000-8179.2023.20221693
Citation: Yan Jin, Xiaojun Yuan, Qiang Zhao, Fu Li, Jianxin Li, Lian Jiang, Jun Lu, Yunpeng Dai, Ping Zhao, Jianghua Zhan, Jie Li, Yanna Cao, Zhanglin Li, Jiaxing Yang, Zhongyuan Li, Daowei Wang, Baocheng Gong, Jie Yan. Summary of the multicenter application of the CCCG-NB-2015 consensus in neuroblastoma[J]. CHINESE JOURNAL OF CLINICAL ONCOLOGY, 2023, 50(9): 433-442. DOI: 10.12354/j.issn.1000-8179.2023.20221693
shu

神经母细胞瘤CCCG-NB-2015共识多中心应用总结

Summary of the multicenter application of the CCCG-NB-2015 consensus in neuroblastoma

    摘要
  • 摘要:
      目的  对中国多中心应用CCCG-NB-2015共识方案诊治的儿童神经母细胞瘤（neuroblastoma，NB）病例进行回顾性分析总结，以期为改进NB诊疗工作提供有力依据。
      方法  回顾性分析2013年3月至2020年7月经统一规范的CCCG-NB-2015共识方案诊治的全国多中心500例NB患儿的临床和预后资料，通过Cox单因素和多因素回归分析明确影响NB预后的关键因素；分别绘制神经元特异性烯醇化酶（neuron-specific enolase，NSE）和乳酸脱氢酶（lactate dehydrogenase，LDH）在预测骨髓转移和复发的ROC曲线，明确NSE和LDH的临床价值；比对分析NB两种不同分期系统和危险度分组系统下患儿的预后差异，着重分析中危组人群的队列特征和预后结局。
      结果  通过单因素分析共确定10个潜在的预后因子，分别为年龄、肿瘤原发部位、INPC分类、骨髓转移、MYCN状态、INSS分期、INRGSS分期、诊断时NSE、LDH水平和影像学定义的危险因素（image-defined risk factors，IDRFs），均P<0.05。Cox多因素分析结果显示与无事件生存率（event-free survival，EFS）密切相关的独立预后因子为INPC分类、骨髓转移、诊断时NSE、LDH水平，与OS密切相关的独立预后因子为NSE、LDH水平及INRGSS分期（均P<0.05）；NSE和LDH可以较好地预测NB骨髓转移、复发等事件发生（均P<0.05)；INSS分期中非4期和4期的3年EFS分别为93.8%和52.6%；转换为INRGSS分期后，L1、L2、MS和M期的3年EFS分别为94.4%、87.2%、87.5%和53.6%。低危组、中危组和高危组3年和5年的EFS分别为97.1%和97.1%、90.0%和87.3%、53.9%和47.3%；其中INSS分期中3期且伴有INPC为UH型的患儿在CCCG-NB-2015共识方案中被归入高危组，其预后差，5年EFS为65.7%（P<0.05），但却在INRG危险度分组中被列入中危组。
      结论  NSE和LDH水平在预测NB患儿骨髓是否转移以及预后评价方面具有重要价值。INRGSS分期为预后的独立影响因子，其较INSS分期在对患儿预后评估方面更具有临床意义，推荐使用INRGSS分期系统；CCCG-NB-2015共识方案各危险度分组治疗下低中危组预后较好，高危组预后较差，建议将来可降低低危组的治疗强度，而对于基于INRG危险度分组治疗的前提下，中危组的治疗建议继续延用CCCG-NB-2015共识中危组方案。

     

    Abstract:
      Objective  : To analyze and summarize the cases of neuroblastoma (NB) in children diagnosed and treated by the multi-center application of CCCG-NB-2015 consensus scheme in China, in order to provide a strong basis for improving the diagnosis and treatment of NB.
      Methods  The clinical and prognostic data of 500 patients with NB from multiple centers nationwide diagnosed and treated by the unified and standardized CCCG-NB-2015 program were retrospectively analyzed from March 2013 to July 2020, and the key factors affecting the prognosis of NB were identified through Cox univariate and multivariate regression analysis. The ROC curves of NSE and LDH in predicting bone marrow metastasis and recurrence were plotted, and the clinical value of neuron-specific enolase (NSE) and lactate dehydrogenase (LDH) was clarified. The prognostic differences of patients under the two different stages of NB and the risk group system were compared, and the cohort characteristics and prognostic outcomes of the cohort population in the intermediate-risk group were analyzed.
      Results  A total of 10 potential prognostic factors were identified by univariate analysis, namely age, tumor primary site, INPC classification, bone marrow infiltration, MYCN gene amplification status, INSS stage, INRGSS stage, NSE and LDH levels at diagnosis, and IDRFs (P<0.05). The results of Cox multivariate analysis showed that the independent prognostic factors closely related to event-free survival (EFS) were INPC classification, bone marrow infiltration, NSE and LDH levels at diagnosis, and the independent prognostic factors closely related to OS were NSE, LDH level and INRGSS stage (P<0.05). NSE and LDH can well predict events such as NB bone marrow metastasis and recurrence (P<0.05). The 3-year EFS of INSS 4 and non-INSS 4 was 93.8% and 52.6% respectively. After conversion to INRGSS staging, the 3-year EFS for L1, L2, MS and M were 94.4%, 87.2%, 87.5% and 53.6%, respectively. The EFS at 3 and 5 years in the low-risk, intermediate-risk and high-risk groups were 97.1% and 97.1%, 90.0% and 87.3%, 53.9% and 47.3%, respectively. Among them, patients with stage INSS 3 with UH of INPC were classified as high-risk group in the CCCG-NB-2015 protocol, and their prognosis was poor, with a 5-year EFS of 65.7% (P<0.05), but mostly classified as intermediate-risk in the INRG risk group.
      Conclusions  NSE and LDH levels are of great value in predicting whether bone marrow metastasis and prognosis evaluation in NB patients. INRGSS staging is an independent prognostic factor and has more clinical significance in assessing prognosis than INSS staging, and the INRGSS staging system is recommended. The prognosis of the low- and medium-risk group was better under the treatment of each risk group of the CCCG-NB-2015 regimen, and the prognosis of the high-risk group was poor. It is suggested that the treatment intensity of low-risk group can be reduced in the future. Under the premise of INRG risk group therapy, the regimen of the intermediate-risk group continued to be CCCG-NB-2015 for the intermediate-risk group.

     

    • HTML全文
    • 参考文献(17)
    • 相关文章
    • 施引文献
  • 资源附件(0)

/

返回文章
返回