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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...

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Autores principales: 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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Korean Cancer Association 2021
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.
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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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