Radiation Therapy (RT) for Diffuse Intrinsic Pontine Glioma (DIPG) in Children

Abstract

Tumours of the brainstem account for approximately 10% to 15% of all central nervous system (CNS) neoplasms in children, with diffuse intrinsic pontine glioma (DIPG) being the most common type. Affected patients with DIPG are mostly children at the age of 5 to 10-years-old. While brainstem gliomas may arise in other parts of the brainstem including the midbrain and medulla oblongata with a more favourable prognosis, pontine location is very frequent with a typically aggressive disease cause leading to a limited lifespan for the affected patients. Patients with DIPG may present with cranial nerve symptoms due to compression and dysfunction of nuclei and tracts located in the pons. A wide spectrum of symptoms may occur including impaired vision and diplopia, nausea and vomiting, headache, impaired alignment of the eyes, gait disturbances, dysarthria, facial asymmetry or weakness, impaired communication with altered levels of consciousness, changes in behaviour, impaired mobility, spasticity, weakness in legs and arms. Brainstem gliomas located at the pons with diffuse and extensive infiltration are typically not amenable for complete surgical resection. In this context, radiation therapy (RT) has traditionally been the mainstay of treatment for DIPG. Optimal radiation dose and fractionation and combined modality management with RT and chemotherapy has been the focus of extensive research over several decades. Herein, we assess the utility of RT for DIPG management in light of the literature.