Suggestions for Escaping The Dark Ages for Pediatric Diffuse Intrinsic Pontine Glioma Treated with Radiotherapy: Analysis of Prognostic Factors From The National Multicenter Study

Purpose This multicenter retrospective study aimed to investigate prognostic factors for survival, encompassing clinical and radiologic features and treatments, in newly diagnosed diffuse intrinsic pontine glioma (DIPG) patients treated with radiotherapy. Methods Patients <30 years of age who underwent radiotherapy as an initial treatment for DIPG between 2000 and 2018 were included; patients who did not undergo an MRI at diagnosis and those with pathologically diagnosed grade I glioma were excluded. We examined medical records of 162 patients collected from 10 participating centers in Korea. The patients’ clinical and radiological variables, molecular and histopathologic data, and treatment response were evaluated to identify the prognosticators for DIPG and estimate survival outcomes. Results The median follow-up period was 10.8 months (interquartile range, 7.5–18.1). The 1- and 2-year overall survival (OS) rates were 53.5% and 19.0%, respectively, with a median OS of 13.1 months. Long term survival rate (≥2 years) was 16.7%, and median OS was 43.6 months. Age (<10 years), poor performance status, treatment before 2010, and post-radiotherapy necrosis were independent prognostic factors related to poor OS in the multivariate analysis. In patients with increased post-radiotherapy necrosis, the median OS was 13.3 months for bevacizumab group and 11.4 months for no-bevacizumab group (P = 0.138). Conclusion Therapeutic strategy for DIPG has remained unchanged over time, and its prognosis remains poor. Our findings suggest that appropriate efforts are needed to reduce the occurrence of post-radiotherapy necrosis. Further well-designed clinical trials are recommended to improve the poor prognosis observed in DIPG patients.