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Clinical and Genomic Characteristics of Adult Diffuse Midline Glioma

Cancer Research and Treatment 2021년 53권 2호 p.389 ~ 398
박창희, 김태민, 배정모, 윤홍석, 김진욱, 최승홍, 이순태, 이주호, 박성혜, 박철기,
소속 상세정보
박창희 ( Park Chang-Hee ) - Seoul National University Hospital Department of Internal Medicine
김태민 ( Kim Tae-MIn ) - Seoul National University Hospital Department of Internal Medicine
배정모 ( Bae Jeong-Mo ) - Seoul National University Hospital Department of Pathology
윤홍석 ( Yun Hong-Seok ) - Seoul National University Hospital Biomedical Research Institute
김진욱 ( Kim Jin-Wook ) - Seoul National University Hospital Department of Neurosurgery
최승홍 ( Choi Seung-Hong ) - Seoul National University Hospital Department of Radiology
이순태 ( Lee Soon-Tae ) - Seoul National University Hospital Department of Neurology
이주호 ( Lee Joo-Ho ) - Seoul National University Hospital Department of Radiation Oncology
박성혜 ( Park Sung-Hye ) - Seoul National University Hospital Department of Pathology
박철기 ( Park Chul-Kee ) - Seoul National University Hospital Department of Neurosurgery

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.

키워드

Diffuse midline glioma; Concurrent chemoradiotherapy; Targeted sequencing

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