DIPG

What it is

Overview

DIPG (Diffuse Intrinsic Pontine Glioma) is a devastating malignant brain tumor arising in the pons — the part of the brainstem that controls vital functions like breathing and heart rate. It primarily strikes children between ages 5 and 10. Because of its location, surgical removal is impossible. Median survival after diagnosis is just 9–11 months, and fewer than 1% of children survive 5 years. DIPG is one of the most urgent unmet needs in pediatric oncology.

Biology

How It Develops

In ~80% of cases, DIPG is driven by a specific mutation: H3K27M — a point mutation in the histone H3 gene that rewires the epigenetic landscape of the cell, silencing tumor suppressor genes across the genome. This mutation is found almost exclusively in pediatric diffuse midline gliomas and is now a diagnostic marker. There is no proven curative treatment.

Warning signs

Symptoms

• —Cranial nerve palsies (double vision, facial weakness, difficulty swallowing)
• —Difficulty walking or balance problems (ataxia)
• —Weakness in arms or legs
• —Morning headaches and vomiting
• —Behavioral or personality changes

Detection

Diagnosis Methods

• —MRI of the brainstem (diagnosis can often be made by imaging alone)
• —Liquid biopsy or tumor biopsy (increasingly done for molecular profiling)
• —H3K27M mutation testing

Medical care

Treatment Options

• —Radiation therapy (palliative — provides temporary relief in ~80% of children)
• —ONC201 (FDA-approved for H3K27M-mutant disease — first targeted therapy)
• —Clinical trials (the standard of care recommendation for all DIPG patients)
• —No curative treatment currently exists

Data

Statistics

Prevention

Risk Factors

• —Age 5–10 (peak incidence)
• —No known modifiable risk factors
• —Sporadic — occurs without family history in nearly all cases

Further reading

Resources