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Clinical and Genomic Characteristics of Adult Diffuse Midline Glioma
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...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Korean Cancer Association
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8053865/ https://www.ncbi.nlm.nih.gov/pubmed/33171023 http://dx.doi.org/10.4143/crt.2020.694 |
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author | Park, Changhee Kim, Tae Min Bae, Jeong Mo Yun, Hongseok Kim, Jin Wook Choi, Seung Hong Lee, Soon-Tae Lee, Joo Ho Park, Sung-Hye Park, Chul-Kee |
author_facet | Park, Changhee Kim, Tae Min Bae, Jeong Mo Yun, Hongseok Kim, Jin Wook Choi, Seung Hong Lee, Soon-Tae Lee, Joo Ho Park, Sung-Hye Park, Chul-Kee |
author_sort | Park, Changhee |
collection | PubMed |
description | 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 PIK3CA(R38S+E545K) and KRAS(G12A) 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. |
format | Online Article Text |
id | pubmed-8053865 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Korean Cancer Association |
record_format | MEDLINE/PubMed |
spelling | pubmed-80538652021-04-29 Clinical and Genomic Characteristics of Adult Diffuse Midline Glioma Park, Changhee Kim, Tae Min Bae, Jeong Mo Yun, Hongseok Kim, Jin Wook Choi, Seung Hong Lee, Soon-Tae Lee, Joo Ho Park, Sung-Hye Park, Chul-Kee Cancer Res Treat Original Article 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 PIK3CA(R38S+E545K) and KRAS(G12A) 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. Korean Cancer Association 2021-04 2020-11-09 /pmc/articles/PMC8053865/ /pubmed/33171023 http://dx.doi.org/10.4143/crt.2020.694 Text en Copyright © 2021 by the Korean Cancer Association https://creativecommons.org/licenses/by-nc/4.0/This is an Open-Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) ) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Original Article Park, Changhee Kim, Tae Min Bae, Jeong Mo Yun, Hongseok Kim, Jin Wook Choi, Seung Hong Lee, Soon-Tae Lee, Joo Ho Park, Sung-Hye Park, Chul-Kee Clinical and Genomic Characteristics of Adult Diffuse Midline Glioma |
title | Clinical and Genomic Characteristics of Adult Diffuse Midline Glioma |
title_full | Clinical and Genomic Characteristics of Adult Diffuse Midline Glioma |
title_fullStr | Clinical and Genomic Characteristics of Adult Diffuse Midline Glioma |
title_full_unstemmed | Clinical and Genomic Characteristics of Adult Diffuse Midline Glioma |
title_short | Clinical and Genomic Characteristics of Adult Diffuse Midline Glioma |
title_sort | clinical and genomic characteristics of adult diffuse midline glioma |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8053865/ https://www.ncbi.nlm.nih.gov/pubmed/33171023 http://dx.doi.org/10.4143/crt.2020.694 |
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