ArticleNeuro-oncology advances
H3K27M-altered diffuse midline glioma: Experience and outcomes from a low-middle income country setting in the contemporary era.
Article in Neuro-oncology advances. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Purpose: H3K27M-altered diffuse midline glioma (DMG) is an aggressive pediatric-type high-grade glioma (HGG) arising from midline structures in the central nervous system. This report summarizes the experience with this disease entity at a tertiary-care comprehensive cancer center in a low-middle income country. Methods: Patients with biopsy-proven H3K27M-altered DMG were identified from prospective database with retrospective data extraction and analysis. Following biopsy/debulking surgery, patients were treated with radiotherapy (RT) with or without temozolomide (TMZ). Progression-free survival (PFS) and overall survival (OS) were calculated using Kaplan-Meier methods and expressed as point estimates with 95% confidence intervals (CI). Results: A total of 225 patients with biopsy-proven H3K27M-altered DMG were identified; survival analysis was restricted to 194 patients with outcome data. Median age at diagnosis was 20 years with 70% patients being ≥12 years of age. At a median follow-up of 12 months, 1-year PFS and OS were 30.8% (95% CI: 24.8%-38%) and 46.8% (95% CI: 40%-54.5%), respectively, yielding median PFS and OS of 7 months (95% CI: 6-10 months) and 12 months (95% CI: 10-14 months), respectively. Leptomeningeal dissemination at initial diagnosis (10.9%) and on follow-up (24.5%) was documented in a substantial proportion of evaluable patients. Female gender, supratentorial location, debulking surgery, administration of RT, and addition of TMZ emerged as prognostic factors for survival. Conclusion: Despite contemporary standard-of-care chemoradiotherapy paradigm, prognosis of H3K27M-altered DMG-an aggressive pediatric-type HGG-remains universally poor with virtually no long-term survivors. Deeper molecular insights and better elucidation of biological underpinnings are required to guide discovery of novel targeted therapeutics.
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