Authors
Daniel C Moreira, Ibrahim Qaddoumi, Susan Spiller, Thomas W Bouldin, Alan Davidson, Nasjla Saba-Silva, Daniel V Sullivan, Ryuma Tanaka, Aaron S Wagner, Matthew Wood, Paul Klimo, Godwin Job, Meenakshi Devidas, Xiaoyu Li, Amar Gajjar, Giles W Robinson, Jason Chiang
Publication date
2024/3/11
Journal
Neuro-oncology
Pages
noae048
Publisher
Oxford University Press
Description
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%). Most tumors were in the cerebral hemispheres (n = 22, 67%). Two …
Total citations
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