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Clinical and Genetic Features of Brainstem Glioma in Adults: A Report of 50 Cases in a Single Center
BACKGROUND AND PURPOSE: Brainstem gliomas (BSGs) in adults are rare brain tumors with dismal outcomes. The aim of this study was to determine the clinical and genetic features in a series of BSGs and their association with the prognosis. METHODS: Fifty patients who underwent a stereotactic biopsy be...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Korean Neurological Association
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8053546/ https://www.ncbi.nlm.nih.gov/pubmed/33835742 http://dx.doi.org/10.3988/jcn.2021.17.2.220 |
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author | Zhou, Chunhui Zhao, Hao Yang, Fan Huangfu, Luokai Dong, Chao Wang, Shuwei Zhang, Jianning |
author_facet | Zhou, Chunhui Zhao, Hao Yang, Fan Huangfu, Luokai Dong, Chao Wang, Shuwei Zhang, Jianning |
author_sort | Zhou, Chunhui |
collection | PubMed |
description | BACKGROUND AND PURPOSE: Brainstem gliomas (BSGs) in adults are rare brain tumors with dismal outcomes. The aim of this study was to determine the clinical and genetic features in a series of BSGs and their association with the prognosis. METHODS: Fifty patients who underwent a stereotactic biopsy between January 2016 and April 2018 at a single institution were collected. Data on clinicopathological characteristics were analyzed and factors associated with patient survival were identified using a Cox regression model. RESULTS: The median age at diagnosis was 55.5 years, and 62% of the patients were male. Glioblastoma (44%) accounted for the largest proportion of BSGs, and oligodendroglioma (2 of 50) was rarely encountered. The IDH mutation (6 of 44) occurred infrequently in astrocytomas, and IDH-mutant tumors harbored both ATRX loss and MGMT promoter methylation at a relatively low level. Wild-type IDH astrocytomas were identified as having high rates of 1p/19q codeletion (5 of 38) and loss of heterozygosity 1p (8 of 38) or 19q (8 of 38) only. In diffuse midline glioma H3K27M mutant, MGMT promoter methylation occurred in three of four cases. Patients were offered radiotherapy and/or concurrent/adjuvant temozolomide chemotherapy, and their median survival time was 13 months. Multivariate analysis revealed that a low tumor grade, absence of tumor enhancement, duration of symptoms ≥3 months, Karnofsky performance status ≥70, and ATRX loss conferred a survival advantage. CONCLUSIONS: Adult BSGs showed different molecular genetic characteristics, but also resembled supratentorial gliomas in their clinical features associated with oncological outcomes. |
format | Online Article Text |
id | pubmed-8053546 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Korean Neurological Association |
record_format | MEDLINE/PubMed |
spelling | pubmed-80535462021-04-29 Clinical and Genetic Features of Brainstem Glioma in Adults: A Report of 50 Cases in a Single Center Zhou, Chunhui Zhao, Hao Yang, Fan Huangfu, Luokai Dong, Chao Wang, Shuwei Zhang, Jianning J Clin Neurol Original Article BACKGROUND AND PURPOSE: Brainstem gliomas (BSGs) in adults are rare brain tumors with dismal outcomes. The aim of this study was to determine the clinical and genetic features in a series of BSGs and their association with the prognosis. METHODS: Fifty patients who underwent a stereotactic biopsy between January 2016 and April 2018 at a single institution were collected. Data on clinicopathological characteristics were analyzed and factors associated with patient survival were identified using a Cox regression model. RESULTS: The median age at diagnosis was 55.5 years, and 62% of the patients were male. Glioblastoma (44%) accounted for the largest proportion of BSGs, and oligodendroglioma (2 of 50) was rarely encountered. The IDH mutation (6 of 44) occurred infrequently in astrocytomas, and IDH-mutant tumors harbored both ATRX loss and MGMT promoter methylation at a relatively low level. Wild-type IDH astrocytomas were identified as having high rates of 1p/19q codeletion (5 of 38) and loss of heterozygosity 1p (8 of 38) or 19q (8 of 38) only. In diffuse midline glioma H3K27M mutant, MGMT promoter methylation occurred in three of four cases. Patients were offered radiotherapy and/or concurrent/adjuvant temozolomide chemotherapy, and their median survival time was 13 months. Multivariate analysis revealed that a low tumor grade, absence of tumor enhancement, duration of symptoms ≥3 months, Karnofsky performance status ≥70, and ATRX loss conferred a survival advantage. CONCLUSIONS: Adult BSGs showed different molecular genetic characteristics, but also resembled supratentorial gliomas in their clinical features associated with oncological outcomes. Korean Neurological Association 2021-04 2021-01-18 /pmc/articles/PMC8053546/ /pubmed/33835742 http://dx.doi.org/10.3988/jcn.2021.17.2.220 Text en Copyright © 2021 Korean Neurological 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 Zhou, Chunhui Zhao, Hao Yang, Fan Huangfu, Luokai Dong, Chao Wang, Shuwei Zhang, Jianning Clinical and Genetic Features of Brainstem Glioma in Adults: A Report of 50 Cases in a Single Center |
title | Clinical and Genetic Features of Brainstem Glioma in Adults: A Report of 50 Cases in a Single Center |
title_full | Clinical and Genetic Features of Brainstem Glioma in Adults: A Report of 50 Cases in a Single Center |
title_fullStr | Clinical and Genetic Features of Brainstem Glioma in Adults: A Report of 50 Cases in a Single Center |
title_full_unstemmed | Clinical and Genetic Features of Brainstem Glioma in Adults: A Report of 50 Cases in a Single Center |
title_short | Clinical and Genetic Features of Brainstem Glioma in Adults: A Report of 50 Cases in a Single Center |
title_sort | clinical and genetic features of brainstem glioma in adults: a report of 50 cases in a single center |
topic | Original Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8053546/ https://www.ncbi.nlm.nih.gov/pubmed/33835742 http://dx.doi.org/10.3988/jcn.2021.17.2.220 |
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