NFM-01. NF105: A PHASE II PROSPECTIVE STUDY OF CABOZANTINIB (XL184) FOR PLEXIFORM NEUROFIBROMAS IN SUBJECTS WITH NEUROFIBROMATOSIS TYPE 1: A NEUROFIBROMATOSIS CLINICAL TRIAL CONSORTIUM (NFCTC) STUDY

BACKGROUNDPlexiform neurofibromas (PNs) are histologically complex peripheral nerve sheath tumors composed of Schwann cells and fibrocytes. Pre-clinical models have demonstrated that altering the microenvironment with molecular targeting therapy may lead to the shrinkage of PN. Here we test the activity of Cabozantinib, a tyrosine kinase inhibitor of KIT, MET, VEGF, and AXL, in adolescents and adults with NF1-associated PN.