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Association of image-defined risk factors with clinical features, tumor biology, and outcomes in neuroblastoma: a single-center retrospective study

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Abstract

Image-defined risk factors (IDRF) in neuroblastoma have been developed to predict tumor resectability and surgical complications; however, the potential prognostic value of IDRF in neuroblastoma has been variably reported. Previous studies did not report the IDRF status separately from the International Neuroblastoma Risk Group (INRG) stage. Moreover, the association between IDRF and clinical and pathological factors has not been discussed further. In this retrospective study, we investigated the clinical and biological features of neuroblastoma at different INRG stages based on IDRF. Event-free survival (EFS) and overall survival (OS) related to the INRG stage were analyzed using log-rank tests, and the prognostic value of the IDRF number and type was also evaluated. Among 72 patients, 182 IDRF at diagnosis were found in 79.2%. The distribution of the INRG stages was 10 L1 (13.9.0%), 25 L2 (34.7%), and 37 M/MS (51.4%). Patients with stage M/Ms had a larger tumor volume, a higher percentage of age ≥ 18 months, elevated lactate dehydrogenase (LDH) level, elevated ferritin level, and a higher percentage of COG high-risk compared with stage L1 and L2 patients. EFS and OS were similar for stage L1 and L2 tumors but were significantly poorer for metastatic disease. However, EFS (P = 0.06) and OS (P = 0.07) were similar for IDRF-negative and positive neuroblastomas. Patients with stage M/Ms with IDRF-positive had poorer EFS (P = 0.001) and OS (P < 0.001) compared with patients in stage L2. An IDRF ≥ 4, vascular IDRF, and infiltrative IDRF of the tumor were significant indicators of poor prognosis.

  Conclusion: Our study indicates that increasing the INRG stages based on IDRF is associated with various unfavorable clinical features of neuroblastoma. The principal determinant of survival in neuroblastoma is the presence of metastatic disease more than IDRF alone at diagnosis. Both the number and type of IDRF have important clinical significance in the protocol planning of neuroblastoma, rather than just considering the absence or presence of IDRF.

What is Known:

• The International Neuroblastoma Risk Group Staging System (INRGSS) now employs image-defined risk factors (IDRFs) to stratify and stage disease.

• The presence of IDRF at diagnosis are associated with higher rates of operative complications and incomplete surgical resection.

What is New:

• The principal determinant of survival from neuroblastoma is the presence of metastatic disease at diagnosis, more than IDRF alone.

• IDRF number and type should also be considered during the diagnosis and treatment planning of neuroblastoma, rather than just considering the absence or presence of IDRF.

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Data availability

The datasets used and/or analyzed during the current study are available from the corresponding author on reasonable.

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Author notes

  1. Dan-dan Yang and Chuan Liu are equal contributions to this work.

Authors and Affiliations

  1. Department of Radiology, the Third People’s Hospital of Chengdu, 82 Qinglong Street, Chengdu, 610031, China

    Dan-dan Yang, Jin Gao, Qiao-jun Hu, Yong Liang & Jian Liu

  2. Department of Radiology, West China Second University Hospital of Sichuan University, Chengdu, China

    Chuan Liu

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Contributions

YDD, LC and GJ designed the study; YDD, LC and HQJ collected the data; YDD and LC reviewed the CT data; LY, LJ,GJ and YDD analyzed and interpreted the data; YDD and LC drafted the manuscript; LC, LJ and GJ contributed to critical revision of the manuscript. All authors have read and approved the final manuscript.

Corresponding author

Correspondence to Jin Gao.

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This was a retrospective study. Consent was obtained from patients’ parent. No finical or nonfinancial benefits have been received or will be received from any party related directly or indirectly to the subject of this article.

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The authors declare that there is no conflict of interest regarding the publication of this paper.

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Communicated by Peter de Winter.

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Yang, Dd., Liu, C., Gao, J. et al. Association of image-defined risk factors with clinical features, tumor biology, and outcomes in neuroblastoma: a single-center retrospective study. Eur J Pediatr 182, 2189–2196 (2023). https://doi.org/10.1007/s00431-023-04899-0

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