Pediatric H3 G34-mutant diffuse hemispheric glioma: clinical, imaging and molecular prognostic factors, MGMT expression, and temozolomide response - PubMed
8 hours ago
- #Temozolomide response
- #MGMT expression
- #Pediatric glioma
- Pediatric H3 G34-mutant diffuse hemispheric glioma (DHG) has poor outcomes with unclear biological resistance mechanisms.
- A retrospective study of 36 pediatric patients (median age 14 years) showed median progression-free survival (PFS) of 0.7 years and overall survival (OS) of 1.8 years.
- Gross total resection (GTR) improved PFS (p = 0.0046).
- 22.6% of patients had gliomatosis cerebri (infiltration of ≥3 brain lobes) at presentation.
- Frontline temozolomide (TMZ) improved PFS (p = 0.0049) in 58.3% of patients.
- Low MGMT expression correlated with better PFS (p = 0.0039) and OS (p < 0.0001).
- MGMT regulation involved gene body/intronic CpG methylation, not promoter methylation.
- PDGFRA alterations were linked to inferior OS (p = 0.0035).
- 81% of patients had post-radiation local (± distant) recurrence.
- Findings support frontline TMZ use and GTR when feasible.