Radiotherapy for Diffuse Intrinsic Pontine Glioma: Insufficient but Indispensable

ABSTRACT

Diffuse intrinsic pontine gliomas (DIPGs) account for 10%–20% of all central nervous system tumors in children and are the leading cause of death in children with brain tumors. Although many clinical trials have been conducted over the past decades, the survival outcome has remained unchanged. Over 90% of children die within 2 years of the diagnosis, and radiotherapy remains the standard treatment to date. To improve the prognosis, hyperfractionated and hypofractionated radiotherapy and/or addition of radiosensitizers have been investigated. However, none of the radiotherapy approaches have shown a survival benefit, and the overall survival of patients with DIPG is approximately 11 months.Here, we comprehensively review the management of DIPG with focus on radiotherapy.