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Diffuse midline glioma, H3 K27-altered (including DIPG): lines of therapy

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4 standard-of-care settings across 3 lines and 1 biomarker subgroup. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.

LineAll comers
Advanced, first line1
Second line2
Other settings1

Advanced, first line

SubgroupSettingApproachProducts and trialsEvidence
All comersNewly diagnosedStereotactic biopsy for molecular diagnosis and trial eligibility, then focal radiotherapy (about six weeks; hypofractionated schedules are non-inferior); steroids for symptoms. Enrolment in a trial such as ACTION (dordaviprone after radiotherapy) is recommended.91

Second line

SubgroupSettingApproachProducts and trialsEvidence
All comersProgressive after radiotherapy, H3 K27M-mutantDordaviprone (ONC201), FDA accelerated approval August 2025 for patients aged one year and older with progressive disease; re-irradiation is an alternative or addition.61
All comersRecurrent, trial-eligibleGD2 CAR-T (phase 1, Stanford and others), convection-enhanced delivery, epigenetic and combination trials through consortium networks.49

Other settings

SubgroupSettingApproachProducts and trialsEvidence
All comersAll stagesEarly palliative care, steroid-sparing strategies, and support for the family; tissue donation at autopsy has been central to research progress.66

Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.