Li-Fraumeni syndrome (LFS) is a rare autosomal dominant tumor predisposition syndrome caused by heterozygous germline mutation or deletion of the I TP53 i tumor suppressor gene on chromosome 17p13. This absence of I ATRX i loss in patient LF-7 (10 years of age at time of glioma diagnosis) is similar to what has been previously reported in sporadic IDH-mutant astrocytomas in teenagers which lack the I ATRX i inactivation that is typical of their IDH-mutant astrocytoma counterparts in adults [[3], [7]]. Instead, five of the tumors harbored somatic biallelic inactivation of the I NF1 i tumor suppressor gene and one harbored multiple I EGFR i activating missense mutations (p.L861Q and p.R252P) in the absence of I EGFR i gene amplification (Supplementary Table 2). [Extracted from the article]