MR Imaging of Pediatric Low-Grade Gliomas: Pretherapeutic Differentiation of V600E Mutation, Fusion, and Wild-Type Tumors in Patients without Neurofibromatosis-1.

Background And Purpose: The prognosis and treatment of pediatric low-grade gliomas is influenced by their molecular subtype. MR imaging remains the mainstay for initial work-up and surgical planning. We aimed to determine the relationship between imaging patterns and molecular subtypes of pediatric low-grade gliomas.

Materials And Methods: This was a retrospective bi-institutional study for patients diagnosed from 2004 to 2021 with pathologically confirmed pediatric low-grade gliomas molecularly defined as fusion, V600E mutant, or wild-type (which is neither V600E mutant nor fusion). Two neuroradiologists, blinded, independently reviewed imaging parameters from diagnostic MRIs, and discrepancies were resolved by consensus. Bivariate analysis was used followed by pair-wise comparison of the Dwass-Steel-Critchlow-Fligner method to compare the 3 molecular subtypes. Interreader agreement was assessed using κ.

Results: We included 70 patients: 30 fusion, 19 V600E mutant, and 21 wild-type. There was substantial agreement between the readers for overall imaging variables (κ = 0.75). fusion tumors compared with V600E and wild-type tumors were larger ( = .0022), and had a greater mass effect ( = .0053), increased frequency of hydrocephalus ( = .0002), and diffuse enhancement ( <.0001). V600E mutant tumors were more often hemispheric (< .0001), appeared more infiltrative ( = .0002), and, though infrequent, were the only group demonstrating diffusion restriction (qualitatively; = .0042) with a lower ADC ratio (quantitatively) ( = .003).

Conclusions: fusion and V600E mutant pediatric low-grade gliomas have unique imaging features that can be used to differentiate them from each other and wild-type pediatric low-grade glioma using a standard radiology review with high interreader agreement. In the era of targeted therapy, these features can be useful for therapeutic planning before surgery.