H3 K27M-Altered Diffuse Midline Gliomas: A Review
Diffuse midline glioma H3 K27M-altered is a recently renamed high-grade glioma in the 2021 World Health Organization (WHO) Classification of Central Nervous System Tumors, previously being labelled diffuse midline glioma H3 K27M-mutant in the 2016 update and diffuse intrinsic pontine glioma prior to...
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| Format: | Article |
| Language: | English |
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Thieme Medical and Scientific Publishers Pvt. Ltd.
2023-08-01
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| Series: | Indian Journal of Neurosurgery |
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| Online Access: | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0043-1771192 |
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| author | Karol Wiśniewski Andrew Ghaly Kate Drummond Andreas Fahlstrӧm |
| author_facet | Karol Wiśniewski Andrew Ghaly Kate Drummond Andreas Fahlstrӧm |
| author_sort | Karol Wiśniewski |
| collection | DOAJ |
| description | Diffuse midline glioma H3 K27M-altered is a recently renamed high-grade glioma in the 2021 World Health Organization (WHO) Classification of Central Nervous System Tumors, previously being labelled diffuse midline glioma H3 K27M-mutant in the 2016 update and diffuse intrinsic pontine glioma prior to 2016. After identification of multiple alterations causing H3 K27 hypomethylation, the definition of this tumor subtype was changed. To further characterize this new entity in both the pediatric and adult population, we conducted a review of the current literature, investigating genetic, epidemiological, clinical, radiological, histopathological, treatment and prognostic characteristics, particularly highlighting the differences between adults and children. This tumor is more common in children, and has a poorer prognosis. Additionally, childhood H3 K27-altered gliomas are more common in the brainstem, but more common in the thalamus in adults. Sadly, limited treatment options exist for these tumors, with radiotherapy the only treatment shown to improve overall survival. |
| format | Article |
| id | doaj-art-0fe3a3c6a41e460a8f395f3afa18b11b |
| institution | Kabale University |
| issn | 2277-954X 2277-9167 |
| language | English |
| publishDate | 2023-08-01 |
| publisher | Thieme Medical and Scientific Publishers Pvt. Ltd. |
| record_format | Article |
| series | Indian Journal of Neurosurgery |
| spelling | doaj-art-0fe3a3c6a41e460a8f395f3afa18b11b2025-08-20T03:54:29ZengThieme Medical and Scientific Publishers Pvt. Ltd.Indian Journal of Neurosurgery2277-954X2277-91672023-08-01120210411510.1055/s-0043-1771192H3 K27M-Altered Diffuse Midline Gliomas: A ReviewKarol Wiśniewski0Andrew Ghaly1Kate Drummond2https://orcid.org/0000-0002-8152-2120Andreas Fahlstrӧm3https://orcid.org/0000-0002-0298-8775Department of Neurosurgery, Royal Melbourne Hospital, Melbourne, AustraliaDepartment of Neurosurgery, Royal Melbourne Hospital, Melbourne, AustraliaDepartment of Neurosurgery, Royal Melbourne Hospital, Melbourne, AustraliaDepartment of Neurosurgery, Royal Melbourne Hospital, Melbourne, AustraliaDiffuse midline glioma H3 K27M-altered is a recently renamed high-grade glioma in the 2021 World Health Organization (WHO) Classification of Central Nervous System Tumors, previously being labelled diffuse midline glioma H3 K27M-mutant in the 2016 update and diffuse intrinsic pontine glioma prior to 2016. After identification of multiple alterations causing H3 K27 hypomethylation, the definition of this tumor subtype was changed. To further characterize this new entity in both the pediatric and adult population, we conducted a review of the current literature, investigating genetic, epidemiological, clinical, radiological, histopathological, treatment and prognostic characteristics, particularly highlighting the differences between adults and children. This tumor is more common in children, and has a poorer prognosis. Additionally, childhood H3 K27-altered gliomas are more common in the brainstem, but more common in the thalamus in adults. Sadly, limited treatment options exist for these tumors, with radiotherapy the only treatment shown to improve overall survival.http://www.thieme-connect.de/DOI/DOI?10.1055/s-0043-1771192diffuse midline gliomaH3.1 or H3.2 K27-mutantH3-wildtype with EZHIP overexpression EGFR-mutantH3.3 K27-mutant |
| spellingShingle | Karol Wiśniewski Andrew Ghaly Kate Drummond Andreas Fahlstrӧm H3 K27M-Altered Diffuse Midline Gliomas: A Review Indian Journal of Neurosurgery diffuse midline glioma H3.1 or H3.2 K27-mutant H3-wildtype with EZHIP overexpression EGFR-mutant H3.3 K27-mutant |
| title | H3 K27M-Altered Diffuse Midline Gliomas: A Review |
| title_full | H3 K27M-Altered Diffuse Midline Gliomas: A Review |
| title_fullStr | H3 K27M-Altered Diffuse Midline Gliomas: A Review |
| title_full_unstemmed | H3 K27M-Altered Diffuse Midline Gliomas: A Review |
| title_short | H3 K27M-Altered Diffuse Midline Gliomas: A Review |
| title_sort | h3 k27m altered diffuse midline gliomas a review |
| topic | diffuse midline glioma H3.1 or H3.2 K27-mutant H3-wildtype with EZHIP overexpression EGFR-mutant H3.3 K27-mutant |
| url | http://www.thieme-connect.de/DOI/DOI?10.1055/s-0043-1771192 |
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