Major efforts are focused on the use of next-generation sequencing techniques, which have identified additional mutations or copy-number alterations of genes such as TP53, SUZ12, EGFR, CDKN2A, and TERT, not present in the benign form of PN, representing alterations associated with advanced progression to the atypical neurofibroma (AN) and MPNST [6,15,16,17,18,19,20,21,22]. Here, SUZ12 is linked to malignant peripheral nerve sheath tumor.