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Treatment and outcome of pediatric intracranial ependymoma after first relapse

  • Clinical Study
  • Published:
Journal of Neuro-Oncology Aims and scope Submit manuscript

Abstract

Introduction

Almost 50% of children with intracranial ependymoma experience disease relapse, and their outcomes are extremely poor. The aim of this study was to investigate optimal salvage treatment for pediatric intracranial ependymoma after the first relapse and to identify prognostic factors affecting survival.

Methods

We conducted a retrospective analysis of 159 children who underwent initial treatment for intracranial ependymoma at Beijing Tiantan Hospital from 2013 to 2017.

Results

Relapse was observed in 73 patients (73/159; 45.9%), with a median age of 7.2 ± 3.5 years old. Molecular subgrouping analysis identified H3K27me3-negative PF-EPNs in 74% of patients, ST-RELA EPNs in 21% of patients, and H3K27me3-positive PF-EPNs in 5% of patients. The 5-year event-free survival (EFS) and overall survival (OS) rates after first relapse were 21.1% (95% CI 16.0–26.2) and 30.5% (95% CI 19.8–30.8), respectively. Patients with GTR at first relapse had higher 5-year EFS and 5-year OS than those with STR (P = 0.031 and P = 0.003) or no surgery (P = 0.007 and P = 0.001). Radiotherapy or re-radiotherapy at first relapse significantly prolonged 5-year EFS and OS (both P < 0.001). Patients with H3K27me3-negative PF-EPN had worse 5-year EFS and OS than those with ST-RELA EPN (P = 0.001 and P = 0.002). Multivariate analysis showed that both tumor resection and radiotherapy at first relapse had independent prognostic significance for survival (all P < 0.05).

Conclusion

Children with recurrent intracranial EPN have poor outcomes, and surgery and radiotherapy at first relapse should be encouraged to improve their prognosis.

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Data availability

The dataset and material are available from the corresponding authors on reasonable request.

Code availability

Not applicable.

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Author information

Authors and Affiliations

  1. Department of Neurosurgery, Beijing Tian Tan Hospital, Capital Medical University, Beijing, China

    Zhi-ming Liu, Zhe Han, Tao Sun, Wei Liu, Jian Gong, Chun-de Li, Fu Zhao & Yong-ji Tian

  2. Department of Neuropathology, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China

    Jun-mei Wang

  3. Department of Radiation Oncology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China

    Bo Li

  4. Department of Neural Reconstruction, Beijing Neurosurgical Institute, Capital Medical University, Beijing, China

    Fu Zhao

Authors
  1. Zhi-ming Liu
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Contributions

Conception and design: FZ, YT; Development of methodology: FZ, YT; Acquisition of data: CL, ZL., TS, WL, ZH; Analysis and interpretation of data: ZL., BL, JW, ZH, FZ; Writing, review, and/or revision of the manuscript: FZ, YT; Administrative, technical, or material support: ZL., CL, JG; Study supervision: FZ, YT, JG.

Corresponding authors

Correspondence to Chun-de Li, Fu Zhao or Yong-ji Tian.

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The authors indicated no potential conflicts of interest.

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All patients or legal guardians provided written informed consent to participate in this study.

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All patients or legal guardians provided written informed consent to publish their data.

Ethical approval

This study was approved by the ethics committee of Beijing Tiantan Hospital, Capital Medical University. The use of the clinical data was conducted in conformity with the institutional review boards (KY 2020-090-02).

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Liu, Zm., Han, Z., Wang, Jm. et al. Treatment and outcome of pediatric intracranial ependymoma after first relapse. J Neurooncol 157, 385–396 (2022). https://doi.org/10.1007/s11060-022-03975-z

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