Back to Search
Start Over
Comprehensive analysis of MYB/MYBL1-altered pediatric-type diffuse low-grade glioma.
- Source :
-
Neuro-oncology [Neuro Oncol] 2024 Jul 05; Vol. 26 (7), pp. 1327-1334. - Publication Year :
- 2024
-
Abstract
- Background: Pediatric-type diffuse low-grade gliomas (pLGG) harboring recurrent genetic alterations involving MYB or MYBL1 are closely related tumors. Detailed treatment and outcome data of large cohorts are still limited. This study aimed to comprehensively evaluate pLGG with these alterations to define optimal therapeutic strategies.<br />Methods: We retrospectively reviewed details of pLGG with MYB or MYBL1 alterations from patients treated or referred for pathologic review at St. Jude Children's Research Hospital. Tumor specimens were centrally reviewed, and clinical data were collated.<br />Results: Thirty-three patients (18 male; median age, 5 years) were identified. Two tumors had MYBL1 alterations; 31 had MYB alterations, MYB::QKI fusion being the most common (n = 10, 30%). Most tumors were in the cerebral hemispheres (n = 22, 67%). Two patients (6%) had metastasis at diagnosis. The median follow-up was 6.1 years. The 5-year event-free survival (EFS) rate was 81.3% ± 8.3%; the 5-year overall survival (OS) rate was 96.4% ± 4.1%. Patients receiving a near-total or gross-total resection had a 5-year EFS of 100%; those receiving a biopsy or subtotal resection had a 5-year EFS rate of 56.6% ± 15.2% (P < .01). No difference in EFS was observed based on location, histology, or molecular alterations. However, the tumors that progressed or metastasized may have distinct methylation profiles with evidence of activation of the MAPK and PI3K/AKT/mTOR pathways.<br />Conclusions: pLGG with MYB/MYBL1 alterations have good outcomes. Our findings suggest that surgical resectability is a crucial determinant of EFS. Further characterization is required to identify optimal treatment strategies for progressive tumors.<br /> (© The Author(s) 2024. Published by Oxford University Press on behalf of the Society for Neuro-Oncology.)
- Subjects :
- Child
Child, Preschool
Female
Humans
Male
Biomarkers, Tumor genetics
Follow-Up Studies
Neoplasm Grading
Prognosis
Proto-Oncogene Proteins
Retrospective Studies
Survival Rate
Trans-Activators genetics
Brain Neoplasms pathology
Brain Neoplasms genetics
Glioma pathology
Glioma genetics
Proto-Oncogene Proteins c-myb genetics
Subjects
Details
- Language :
- English
- ISSN :
- 1523-5866
- Volume :
- 26
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- Neuro-oncology
- Publication Type :
- Academic Journal
- Accession number :
- 38466086
- Full Text :
- https://doi.org/10.1093/neuonc/noae048