The EGFR inhibitor erlotinib was the first targeted agent used in a histology-specific phase II trial for MPNST [96], based on the compelling preclinical observation that EGFR amplification was observed in MPNSTs and that Nf1/p53 murine MPNST were stimulated by EGF and inhibited by EGFR inhibitors [33]. Here, NF1 is linked to malignant peripheral nerve sheath tumor.