Low-grade gliomas (LGGs) are a diverse group of tumors that accounts for about 30% of CNS primary tumors in children. Management of these tumors, which include surgery and adjuvant chemotherapy, depend largely on the age of the patient and location of these tumors. Recent advances in molecular characterization of RAS/RAF/MAPK pathway have improved our understating of these tumors and provided promising results for treatment. Ninety percent of pilocytic astrcoytoms have BRAF-KIAA1549 fusion gene while 10% show BRAFV600E mutation. Other glioneuronal tumors like gangliogliomas and pleiomorphic xanthoastrocytomas harbor also the BRAFV600E mutation. In our case-based presentation we will show that the presence of BRAFV600E mutation results in favorable response on imaging compared to those cases with BRAF fusion when treated with BRAF inhibitors and MEK inhibitors. The treatment response manifests with reduction in the size of the tumor, decrease in its T2 signal intensity and reduction in enhancement. Some tumors show interval reductions in the size of their cystic components while others show persistent or interval development of small cysts. The imaging response is quite early and dramatic after the initiation of therapy in tumors with BRAFV600E mutation.
No datasets are available for this submission.