A genetic mouse model of malignant peripheral nerve sheath tumor with postnatal Nf1 and p53 loss recapitulates the histology and transcriptome of human tumors

We established a new genetically engineered mouse (GEM) model of malignant peripheral nerve sheath tumors (MPNST) based on postnatal deletion of a Nf1;Trp53 cis-conditional allele by the tamoxifen-inducible Plp-CreER (NP-Plp). We also generated two Lats1;2 conditional knockout models by using Nestin-Cre (Lats-Nes) and Plp-CreER (Lats-Plp), both of which also develop tumors similar to MPNST (GEM-PNST). To evaluate these models, transcriptome analyses were performed to compare these models and with human MPNST, plexiform neurofibromas (PNF), and neurofibromas (NF). Overall design: Transcriptome comparison of various mouse models of maligant peripheral nerve sheath tumor (NP-Plp, NPcis, Lats-Nes, and Lats-Plp) and human MPNST, PNF, and NF tumors.