Using a genetically engineered Nf1 floxed, Cdkn2a/Arf floxed, and PostnCre mouse model that triggered conditional knockout in the nerve crest derived Schwann cell lineage, Rhodes et al. created one of first models that mimic the human malignant transformation from plexiform to atypical neurofibroma, which eventually developed MPNST with a high penetrance [169]. Here, CDKN2A is linked to malignant peripheral nerve sheath tumor.