Effective sorafenib treatment response in a panel of genomically-characterized malignant peripheral nerve sheath tumor orthoxenograft models

ABSTRACT: Background: Malignant peripheral nerve sheath tumors (MPNST) are soft-tissue sarcomas that can arise either sporadically or in association with neurofibromatosis type 1 (NF1). These aggressive malignancies confer poor survival, with no effective therapy available. Methods: We generated five patient-derived MPNST orthoxenograft models (three NF1-related and two sporadic) and performed an exhaustive histological and molecular characterization of primary MPNSTs and their corresponding orthoxenografts. Finally, orthoxenografts models were used as an in vivo pre-clinical platform to test several treatment strategies. Results: MPNST orthoxenografts recapitulate the histopathological properties and preserve the genomic and transcriptomic status of their parental primary tumors. Additionally, they

ORGANISM(S): Homo sapiens

SUBMITTER: Bernat Gel Moreno 

PROVIDER: E-GEOD-60082 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress