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.
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