Diffuse intrinsic pontine glioma

(DIPG) / Diffuse midline glioma (DMG), H3 K27-altered/ Brainstem glioma / pontine glioma

A diffusely infiltrating glioma centered in the ventral pons that is clinically inoperable because of location and infiltration of critical brainstem structures.

Most pediatric DIPGs carry histone H3 K27-altering mutations and are aggressive.

Primarilv a disease of children, median age 5-10 vears . Clinical progression is usually inevitable despite treatment.

Typical clinical presentation

○ Rapidly progressive brainstem dysfunction over weeks.
○ Cranial nerve deficits: facial weakness, diplopia (abducens palsy), dysphagia.
○ Long-tract signs: weakness, hyperreflexia.
○ Ataxia and gait disturbance.
○ Bulbar symptoms (speech/swallowing) in advanced disease.
○ Headache and vomiting from obstructive hydrocephalus may occur.