Table 1.
BRAF alteration | BRAF location | |||
---|---|---|---|---|
Cerebellum | Supratentorial | Non-cerebellar infratentorial | Optic nerve | |
B–K fusion | 74.2 % | 33.2 % | 50.6 % | 54.2 % |
V600E | 2.9 % | 17.9 % | 1.6 % | 15.0 % |
BRAF alteration | BRAF histology | |||||
---|---|---|---|---|---|---|
PA | PMA | PXA | GG | DA | HGG | |
B–K fusion | 66.6 % | 50.0 % | 0.0 % | 21.2 % | 11.9 % | 0.0 % |
V600E | 5.7 % | 7.7 % | 73.8 % | 19.2 % | 9.7 % | 13.1 % |
At least half of all posterior fossa/infratentorial and optic nerve low-grade gliomas have a B–K fusion, whereas only one third of all supratentorial non-optic nerve tumors have this alteration. In contrast, BRAFV600E mutation is most common in the supratentorium, although still half as frequent as the B–K fusion in this region. BRAF fusions are more associated with PAs and PMAs than any other histotype. Although some studies have identified B–K fusions in non-PA tumors, current thinking is that such tumors may be better classified as a PA or PMA, even if they resemble GGs or DAs. V600E mutations show less restriction in histotype, but are most common in PXAs and GGs. Although not listed in the Table, FAM131B:BRAF, SRGAP3:RAF1, and BRAFins598T mutations occur in 1%–2% of all PAs. These data were extracted and compiled from an aggregate of over 700 pediatric gliomas published by multiple groups (5–11, 15, 17, 20, 22, 33, 34, 45). PA = pilocytic astrocytoma; PMA = pilomyxoid astrocytoma; PXA = pleomorphic xanthoastrocytoma; GG = ganglioglioma; DA = grade II diffuse astrocytoma; HGG = high-grade glioma.