H3K27M mutant glioma: Disease definition and biological underpinnings.
| Autor: | Saratsis AM; Advocate Aurora Healthcare, Chicago, Illinois, USA., Knowles T; Claremont McKenna College, Claremont, California, USA., Petrovic A; DMG Research Center, Department of Oncology, University Children's Hospital, University of Zürich, Zürich, Switzerland., Nazarian J; Research Center for Genetic Medicine, Children's National Health System, Washington, District of Columbia, USA.; Brain Tumor Institute, Children's National Health System, Washington, District of Columbia, USA.; DMG Research Center, Department of Pediatrics, University Children's Hospital, University of Zurich, Zürich, Switzerland. |
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| Jazyk: | angličtina |
| Zdroj: | Neuro-oncology [Neuro Oncol] 2024 May 03; Vol. 26 (Supplement_2), pp. S92-S100. |
| DOI: | 10.1093/neuonc/noad164 |
| Abstrakt: | High-grade glioma (HGG) is the most common cause of cancer death in children and the most common primary central nervous system tumor in adults. While pediatric HGG was once thought to be biologically similar to the adult form of disease, research has shown these malignancies to be significantly molecularly distinct, necessitating distinct approaches to their clinical management. However, emerging data have shown shared molecular events in pediatric and adult HGG including the histone H3K27M mutation. This somatic missense mutation occurs in genes encoding one of two isoforms of the Histone H3 protein, H3F3A (H3.3), or HIST1H3B (H3.1), and is detected in up to 80% of pediatric diffuse midline gliomas and in up to 60% of adult diffuse gliomas. Importantly, the H3K27M mutation is associated with poorer overall survival and response to therapy compared to patients with H3 wild-type tumors. Here, we review the clinical features and biological underpinnings of pediatric and adult H3K27M mutant glioma, offering a groundwork for understanding current research and clinical approaches for the care of patients suffering with this challenging disease. (© The Author(s) 2023. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.) |
| Databáze: | MEDLINE |
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