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A nomogram for predicting recurrence-free survival of intermediate and high-risk neuroblastoma

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Abstract

This study aimed to confirm the independent risk factors for recurrence-free survival (RFS) in intermediate and high-risk neuroblastoma (NB) patients and set up an effective nomogram model for predicting the recurrence of NB. A total of 212 children with intermediate- and high-risk neuroblastoma, who had ever achieved complete remission (CR) or very good partial remission (VGPR) after standardized treatment in this hospital, were chosen as study objects. After retrospective analysis of the clinical data, Cox regression model was used to explore the factors related to the recurrence of neuroblastoma, to determine the variables to construct the Nomogram. The consistency index would predict the accuracy of this nomogram. RFS rate in 1-year, 3-year, 5-year, and 10-year was 0.811, 0.662, 0.639, and 0.604, respectively. Children with MYCN amplification had a higher neuron-specific enolase (NSE) value (P = 0.031) at the initial diagnosis than MYCN non-amplification. The univariate analysis predicted that increased vanillylmandelic acid (VMA) and NSE value and dehydrogenase (LDH) > 1000 U/L were important adverse factors for the recurrence of NB. Multivariate analysis demonstrated that age at diagnosis, tumor localization, MYCN state, histologic subtype, and tumor capsule were significantly associated with RFS (all P values < 0.05). Nomograms were established for predicting the recurrence of NB according to the Cox regression analysis. Internal verification by the Bootstrap method showed that the prediction of the nomogram’s consistency index (C-index) was 0.824 (P = 0.023).

   Conclusion: Age at diagnosis, tumor localization, MYCN state, histologic category, and tumor capsule were independent risk factors for the recurrence of NB. The nomogram model could accurately predict the recurrence of children with neuroblastoma.

What is Known:

• The prognoses of neuroblastoma (NB) could vary greatly due to the high heterogeneity, the 5-year survival rate of low-risk NB exceeded 90%, while the 5-year survival rate of children in the intermediate and high-risk groups was not satisfactory..

What is New:

• Increased vanillylmandelic acid (VMA) and neuron-specific enolase (NSE) value, and lactate dehydrogenase (LDH)>1000U/L were important adverse factors for the recurrence of NB.

• NSE value was more valuable for predicting NB recurrence.

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Acknowledgements

The authors wish to thank the patients and the staff in the Department of Pediatric Hematology/Oncology, Xinhua Hospital Affiliated to Medicine School of Shanghai Jiaotong University.

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Author notes

  1. Quan Sun and Yanmin Chen contributed equally to this work.

Authors and Affiliations

  1. Department of Pediatric Hematology/Oncology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, People’s Republic of China

    Quan Sun, Yanmin Chen, Qianya Jin & Xiaojun Yuan

Authors
  1. Quan Sun
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  2. Yanmin Chen
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  4. Xiaojun Yuan
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Contributions

All the authors contributed to the study conception and design. Data collection, follow-up, and analysis were performed by Quan Sun, Yanmin Chen, Qianya Jin, and Xiaojun Yuan. The first draft of the manuscript was written by Quan Sun, and all the authors commented on previous versions of the manuscript. All the authors read and approved the final manuscript.

Corresponding author

Correspondence to Xiaojun Yuan.

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The ethics approval was granted by the ethics committee of Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine. This study was conducted in accordance with the principles of the Declaration of Helsinki.

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The authors declare no competing interests.

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Communicated by Peter de Winter

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Sun, Q., Chen, Y., Jin, Q. et al. A nomogram for predicting recurrence-free survival of intermediate and high-risk neuroblastoma. Eur J Pediatr 181, 4135–4147 (2022). https://doi.org/10.1007/s00431-022-04617-2

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