NF1 and malignant peripheral nerve sheath tumor: In addition, other events such as TP53 mutation have also been noted in biologically aggressive peripheral nerve sheath tumors.30–32,37 While infrequent, TP53-altered MPNST have worse clinical outcomes relative to TP53-wildtype cases.38,39 In zebrafish, tp53-altered lines developed MPNST and in mice, homozygous inactivation of Nf1 and Tp53 in combination with Suz12 constitute well-established models of MPNST.34,40,41,42TP53 inactivation may also drive chromosomal instability in altered tumors, although the relation of TP53 mutations to aneuploidy in MPNST remains poorly characterized.43,44