Re-Irradiation for the Progressive Pediatric Diffuse Intrinsic Pontine Glioma: A Report on 109 Children From a Single Center

Abstract

Background

Diffuse intrinsic pontine glioma (DIPG) is a challenging pediatric tumor that frequently progresses within the first year following local radiotherapy. However, several small studies have suggested that re-irradiation may improve quality of life and extend overall survival.

Patients and Methods

This retrospective study included 109 children who experienced disease progression ≥3 months after their initial radiotherapy, and subsequently received re-irradiation at a single institution. These patients were compared with a cohort of 60 children, meeting the same criteria, who were treated before adopting the re-irradiation policy and received only the best supportive care (BSC). Most of the re-irradiated children (94%) received first radiation dose as hypofractionation (39 Gy/13 fractions).

Results

The re-irradiation group demonstrated significantly higher overall survival (OS) rates after the first progression, with a 6-month OS of 42% (95% CI: 34%–53%) compared to 16% (95% CI: 8.9%–32%) in the BSC group (p < 0.001). Re-irradiation reduced the hazard of death by more than half (HR = 0.45, p < 0.001). Clinical response (p < 0.001) and radiological response (p = 0.016) were significant predictors of improved survival. While the time from initial radiotherapy to progression (p = 0.059) and higher re-irradiation doses (p = 0.074) were associated with improved OS, these factors did not reach statistical significance but may represent potential prognostic indicators.

Conclusion

Re-irradiation improved the OS in children with progression of DIPG and alleviated their signs and symptoms.