Introduction
The last decades have witnessed a progressive paradigm shift in the non-surgical management of pediatric low-grade gliomas (PLGG). While radiation was historically the standard treatment, its use has progressively decreased with the development of chemotherapy strategies that have shown the possibility to delay or avoid radiotherapy in most patients1. A new shift is currently happening with evidence that alterations within the mitogen-activated protein kinase (MAPK) pathway affect most PLGGs and represent potential therapeutic targets2. Phase I and II trials have shown the efficacy of BRAF and MEK inhibitors in recurrent and/or refractory PLGG34. Response rates to these agents are promising and appear to be superior to those observed with chemotherapy, and clinical trials are ongoing to compare the efficacy of the new agents with standard chemotherapy in treatment naïve patients 5. However, a number of questions remain unanswered regarding these new compounds, and in particular with regard to the management of patients who fail to respond or progress while treated with MEK or BRAF inhibitors. Herein we describe a patient with BRAFV600E mutated PLGG who progressed on a combination of dabrafenib and tramatenib. This patient eventually responded to a chemotherapeutic regimen consisting of thioguanine, procarbazine, lomustine and vincristine (TPCV) resulting in reversal of life-threatening symptoms.