Pediatric high-grade gliomas in patients with neurofibromatosis type 1-A collaborative cohort study from the SIOPE HGG/DIPG working group - PubMed
3 days ago
- #pediatric oncology
- #neurofibromatosis
- #high-grade glioma
- Study focused on pediatric high-grade gliomas (HGG) in patients with neurofibromatosis type 1 (NF1).
- Retrospective cohort study identified 29 pediatric patients with NF1-associated HGG.
- Median age at diagnosis was 11 years, with most tumors arising outside the optic pathway.
- Molecular analysis showed enrichment of alterations in CDKN2A, TP53, and ATRX.
- Survival rates were as poor as in sporadic HGG patients.
- No significant difference in prognosis between upfront and delayed radiotherapy.
- Adjuvant MEK inhibitor treatment and delayed radiotherapy require further investigation.