In both mouse and human MPNST, mutations or copy number alterations in genes such as TP53, CDKN2A, EGFR, and SUZ12 have all been reported as secondary cooperating mutations facilitating malignant progression.11–15 In this regard, alterations in TP53, EGFR, and SUZ12 are common in MPNST. This evidence concerns the gene CDKN2A and malignant peripheral nerve sheath tumor.