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Diffuse Midline Glioma — SCE Medical Oncology MCQ

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HardCNS TumoursDiffuse Midline GliomaSCE Medical Oncology

A 55-year-old man with a diffuse midline glioma (DMG, formerly DIPG in children) diagnosed on biopsy showing H3K27M mutation asks about treatment options. What is the prognosis and standard treatment?

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Correct answer: D — H3K27M-mutant diffuse midline gliomas have a very poor prognosis

H3K27M-mutant diffuse midline gliomas (thalamus, brainstem, spinal cord) are classified as WHO grade 4 regardless of histological features. Prognosis is poor (median survival ~9-15 months in adults, ~11 months in children). Standard treatment is radiotherapy (54-59.4 Gy) ± temozolomide (limited evidence in DMG, extrapolated from GBM). Clinical trial enrolment is strongly encouraged. Biopsy (stereotactic) is performed for molecular confirmation. ONC201 (dordaviprone, a selective dopamine receptor D2 antagonist) has shown promising early data in H3K27M-mutant gliomas.

Reference: https://www.nice.org.uk/guidance/conditions-and-diseases/cancer