Furthermore, the mutational profile of NF1-pLGG was distinct from NF1-high grade glioma (HGG), which instead harbored alterations in TP53, CDKN2A and ATRX. Therefore, obtaining a biopsy from, at minimum, patients deemed higher risk, may prove valuable in identifying patients that require refined and/or novel treatments and distinguishing them from NF1-HGG, particularly in adults. Here, ATRX is linked to central nervous system cancer.