Diffuse Intrinsic Pontine Glioma

Diffuse intrinsic pontine glioma (DIPG) is a rare pediatric brain tumor that carries a very poor prognosis and lacks any effective treatment. Most seen in children, DIPG can also affect young adults. Presenting symptoms are often related to disruption of neural tracts traveling through the brainstem as patients present acutely with neuromuscular deficits, cranial nerve palsies, or signs of cerebellar involvement. Various epigenetic and genetic mutations have been documented, with mutations in the histone 3 protein family as well as the ACVR1 gene being heavily implicated. Diagnosis is typically made with the typical constellation of clinical presentation and imaging findings, although biopsy of the lesion may be utilized more in the future. While treatment centers around radiation therapy, it has not been shown to be drastically beneficial, and more research needs to be done to find more effective targeted and effective therapies.