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, NF1 is linked to plexiform neurofibroma.