During the preparation of this manuscript, individual cases of WHO grade II PXA with BRAF V600E mutation have been reported with one occurring in the temporal lobe of a 36-year-old woman [39], one in the cerebral cortex of a six-year-old girl [41] and one in the temporal lobe of a 16 year old boy with velocardiofacial syndrome [42]. This evidence concerns the gene BRAF and 22q11.2 deletion syndrome.