The genetic landscape of human malignant peripheral nerve sheath tumors (MPNST) reveals recurrent themes of Ras/Raf pathway activation and impairment of DNA repair and P53 regulation resulting from mutations in NF1 (a hallmark of the inherited disorder neurofibromatosis type 1 which confers increased risk of MPNST), BRAF and CDKN2A/B (89–91). The gene discussed is NF1; the disease is malignant peripheral nerve sheath tumor.