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Purpose <p>To investigate the clinical, surgical, and molecular features and outcomes of adult patients with diffuse midline glioma (DMG), H3 K27-altered, treated with surgical resection.</p> Methods <p>We retrospectively analyzed 52 adult patients with cerebral H3 K27-altered DMG who underwent surgical resection. Next-generation sequencing (NGS) was performed on a subset (<i>n</i> = 20) to characterize the genomic landscape. Survival outcomes were evaluated using Kaplan-Meier analysis and Cox proportional hazards models.</p> Results <p>The median overall survival (OS) was 17.8 months. Genomic profiling revealed a distinct molecular landscape dominated by H3F3A mutations, with high-frequency co-occurring TP53 mutation. Crucially, we observed a significant enrichment of mutations within the RTK/RAS/PI3K signaling pathway, primarily driven by NF1, FGFR1, and PIK3CA. In the multivariable Cox regression model, postoperative adjuvant radiotherapy (RT + TMZ) emerged as the sole independent protective factor for overall survival (HR = 0.12, <i>P</i> &lt; 0.001). Conversely, the extent of resection (RANO EOR Class) and molecular targeted therapy did not reach independent statistical significance, despite demonstrating favorable survival trends in univariable analyses.</p> Conclusion <p>Adult H3 K27-altered DMG represents a biologically unique entity with distinct genomic alterations. While postoperative radiotherapy (RT + TMZ) remains the irreplaceable prognostic cornerstone, maximal safe resection provides an essential cytoreductive foundation. Future paradigms should integrate safe resection and targeted agents into adult-tailored, multi-modal treatment frameworks.</p>

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Clinical, surgical, and molecular characteristics and outcomes of adult patients with diffuse midline glioma, H3 K27-altered, treated with surgical resection

  • 期刊论文

  • 发表日期：

    2026年7月2日

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  • Jinfeng Lin,
  • Woheng Tang,
  • Kaichen Li,
  • Zhuoyang Chen,
  • Huayang Zhang,
  • Zhi Ye,
  • Zhiyong Li,
  • Shanqiang Qu,
  • Guanglong Huang,
  • Guo-zhong Yi

摘要

Purpose

To investigate the clinical, surgical, and molecular features and outcomes of adult patients with diffuse midline glioma (DMG), H3 K27-altered, treated with surgical resection.

Methods

We retrospectively analyzed 52 adult patients with cerebral H3 K27-altered DMG who underwent surgical resection. Next-generation sequencing (NGS) was performed on a subset (n = 20) to characterize the genomic landscape. Survival outcomes were evaluated using Kaplan-Meier analysis and Cox proportional hazards models.

Results

The median overall survival (OS) was 17.8 months. Genomic profiling revealed a distinct molecular landscape dominated by H3F3A mutations, with high-frequency co-occurring TP53 mutation. Crucially, we observed a significant enrichment of mutations within the RTK/RAS/PI3K signaling pathway, primarily driven by NF1, FGFR1, and PIK3CA. In the multivariable Cox regression model, postoperative adjuvant radiotherapy (RT + TMZ) emerged as the sole independent protective factor for overall survival (HR = 0.12, P < 0.001). Conversely, the extent of resection (RANO EOR Class) and molecular targeted therapy did not reach independent statistical significance, despite demonstrating favorable survival trends in univariable analyses.

Conclusion

Adult H3 K27-altered DMG represents a biologically unique entity with distinct genomic alterations. While postoperative radiotherapy (RT + TMZ) remains the irreplaceable prognostic cornerstone, maximal safe resection provides an essential cytoreductive foundation. Future paradigms should integrate safe resection and targeted agents into adult-tailored, multi-modal treatment frameworks.