Cancer Res Treat.  2021 Apr;53(2):389-398. 10.4143/crt.2020.694.

Clinical and Genomic Characteristics of Adult Diffuse Midline Glioma

Affiliations
  • 1Department of Internal Medicine, Seoul National University Hospital, Seoul, Korea
  • 2Cancer Research Institute, Seoul National University, Seoul, Korea
  • 3Department of Pathology, Seoul National University Hospital, Seoul, Korea
  • 4Biomedical Research Institute, Seoul National University Hospital, Seoul, Korea
  • 5Department of Neurosurgery, Seoul National University Hospital, Seoul, Korea
  • 6Department of Radiology, Seoul National University Hospital, Seoul, Korea
  • 7Department of Neurology, Seoul National University Hospital, Seoul, Korea
  • 8Department of Radiation Oncology, Seoul National University Hospital, Seoul, Korea

Abstract

Purpose
The treatment outcomes and genomic profiles of diffuse midline glioma (DMG) in adult patients are rarely characterized. We performed a retrospective study to evaluate the clinicogenomic profiles of adult patients with brain DMG.
Materials and Methods
Patients aged ≥ 18 years diagnosed with brain DMG at Seoul National University Hospital were included. The clinicopathological parameters, treatment outcomes, survival, and genomic profiles using 82-gene targeted next-generation sequencing (NGS) were analyzed. The 6-month progression-free survival (PFS6) after radiotherapy and overall survival (OS) were evaluated.
Results
Thirty-three patients with H3-mutant brain DMG were identified. The median OS from diagnosis was 21.8 months (95% confidence interval [CI], 13.2 to not available [NA]) and involvement of the ponto-medullary area tended to have poor OS (median OS, 20.4 months [95% CI, 9.3 to NA] vs. 43.6 months [95% CI, 18.2 to NA]; p=0.07). Twenty-four patients (72.7%) received radiotherapy with or without temozolomide. The PFS6 rate was 83.3% (n=20). Patients without progression at 6 months showed significantly prolonged OS compared with those with progression at 6 months (median OS, 24.9 months [95% CI, 20.4 to NA] vs. 10.8 months [95% CI, 4.0 to NA]; p=0.02, respectively). Targeted NGS was performed in 13 patients with DMG, among whom nine (69.2%) harbored concurrent TP53 mutation. Two patients (DMG14 and DMG23) with PIK3CAR38S+E545K and KRASG12A mutations received matched therapies. Patient DMG14 received sirolimus with a PFS of 8.4 months.
Conclusion
PFS6 after radiotherapy was associated with prolonged survival in adult patients with DMG. Genome-based matched therapy may be an encouraging approach for progressive adult patients with DMG.

Keyword

Diffuse midline glioma; Concurrent chemoradiotherapy; Targeted sequencing
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