Comprehensive analysis of MYB/MYBL1-altered pediatric-type diffuse low-grade glioma.

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.

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.

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%)

SUBMITTER: Moreira DC 

PROVIDER: S-EPMC11226882 | biostudies-literature | 2024 Jul

REPOSITORIES: biostudies-literature