Systematic review of studies of diffuse midline H3K27-altered gliomas in the spinal cord

Systematic review of published studies evaluated clinical features, management, and outcomes of diffuse midline H3K27-altered gliomas of the spinal cord (DMG-SCs). The review included 39 studies with 279 patients (61% male, average age 32 years). Average median overall survival was 24 months (range 13-40) with a median progression-free survival of 14 months. Despite the recent WHO 2021 grade 4 classification for all DMGs, there was a longer average median overall survival for historical WHO grades 2 or 3 compared to grade 4 (32 versus 23 months; p=0,009). The authors conclude that outcomes for DMG-SCs are poor overall but appear to be favorable compared to intracranial DMGs.