Table 2. Summary table of pediatric high-grade gliomas.
*Neoplasm | Distinct imaging features | Locations | Histology and molecular | Grade (WHO) | Outcome |
---|---|---|---|---|---|
**Diffuse Midline Glioma (DMG), H3K27M | Midline tumor with an infiltrative pattern. When arising from pons causes a diffuse enlargement of this structure “engulfing” anteriorly the basilar artery. Subtle enhancement (linear or punctate) may be seen. Areas of necrosis only in the late stage or after treatment |
Brainstem, particularly the pons, but may arise/infiltrate the basal ganglia, thalami, all parts of the brainstem and spinal cord | GFAP variable, S100+, H3-K27M | IV | Poor outcome |
High Grade Glioma, H3G34 | Heterogeneous features. Infiltrative tumors, with gliomatosis appearance, accompanied by calcification, areas of liquefaction/necrosis and DWI restriction are described | Hemispheric > infratentorial | GFAP variable, S100+, H3.3G34R/V | IV | Poor outcome |
*, High grade gliomas are more often infiltrative hypointense on T1WI and hyperintense on T2WI with variable components of restriction on DWI; **, DMG with bithalamic involvement may lack H3 mutation but present with EGFR mutations which may confer selective vulnerability to EGFR kinase inhibitors.