Our panel of experts highlights the most important research articles across the spectrum of topics relevant to the field of CNS oncology

摘要

The new WHO classification for brain tumors [1]has introduced some new entities like midlineglioma with histone 3 mutation that, as pediatricneuro-oncologists, we have to be aware of properclarification of prognosis with patients and parentsand adequate treatment comprising at leastradiation therapy as for grade 4 glioma. Thispaper reports on the search for BRAF V600Eand H3 K27M mutations in a series of 54 pediatricmidline grade I gangliogliomas (midlineGG) to determine the frequency of double mutationsand its relevance for prognosis. While it iswell known that GG is a grade I tumor characterizedby alterations in the MAPK pathway,including BRAF, the occurrence of the secondmutation was not reported so far. The frequencyof the co-occurrence of H3F3A/BRAF mutationsat diagnosis was 9.3% and the H3F3Amutation was never singularly found. Of the fivepatients harboring both mutations in their GG,only one died due to progressive disease evenwhen nobody had received adjuvant treatmentafter surgery. Despite the presence of H3 K27Mmutation, these cases maintained the good prognosisof GG grade 1 and, the authors suggest,they should not have received any further treatmentafter surgery even if molecular findingswere known at diagnosis. The interaction of thetwo different mutations could be a key of theswitching off of the negative prognosis correlatedto H3K27 mutation.