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MYB Alterations in Angiocentric Gliomas
Isabela Peña Pino1, A Yohan Alexander1, Sanjay Dhawan1
1Department of Neurosurgery and Center for Skull Base and Pituitary Surgery, University of Minnesota, Minneapolis, Minnesota, USA.
MYB alterations are common in angiocentric glioma, with MYB::QKI fusions being the most frequent. This review details their clinical features and highlights MYB fusions as key drivers in neuro-oncologic diseases, suggesting therapeutic potential.
Area of Science:
- Neuro-oncology
- Molecular Pathology
- Genetics
Background:
- Angiocentric glioma is a rare brain tumor.
- MYB gene alterations are increasingly recognized as key drivers in various cancers.
- Understanding MYB alterations in angiocentric glioma is crucial for diagnosis and treatment.
Purpose of the Study:
- To systematically review MYB alterations in angiocentric glioma.
- To define the clinical characteristics associated with these alterations.
- To present a novel MYB mutation in an angiocentric glioma case.
Main Methods:
- Systematic literature review following PRISMA guidelines.
- Searched PubMed and Web of Science (2010-October 2024).
- Included 14 studies with 114 patients; analyzed one novel case.
Main Results:
- The mean patient age was 10.3 years, with 60% males.
- MYB::QKI fusion was the most common alteration (68%).
- Other MYB alterations and cerebral cortex location were frequently observed.
Conclusions:
- This study characterizes angiocentric glioma demographics, location, and MYB fusion patterns.
- MYB fusions are important in neuro-oncologic diseases, including adenoid cystic carcinomas.
- Further research into MYB fusions as prognostic markers and therapeutic targets is warranted.
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