Neoadjuvant Nivolumab Plus Ipilimumab for Newly Diagnosed Malignant Peripheral Nerve Sheath Tumor

Inclusion Criteria: * Histologically confirmed diagnosis of atypical neurofibromatous neoplasms of uncertain biologic potential (ANNUBP), low grade malignant peripheral nerve sheath tumor (MPNST) or high grade MPNST in accordance with the Miettinen et al diagnostic criteria via biopsy * Plexiform neurofibroma or other tumors such as optic pathway glioma, other low-grade glioma or other neoplasm in addition to the ANNUBP, low grade MPNST or high grade MPNST that is stable (has not required treatment in the last 12 months and is not anticipated to need treatment in the next 12 months) * Measureable disease by RECIST criteria in at least one site. * Karnofsky Performance Scale ≥ 60% * No contraindications for Nivolumab or Ipilimumab * Normal organ and marrow function on routine laboratory tests * Evidence of post-menopausal status or negative urinary/serum pregnancy test for female pre-menopausal subjects. Women will be considered post-menopausal if they have been amenorrheic for 12 months without an alternative medical cause * Ability to understand and willingness of sign consent form * Willingness to comply with the protocol for the duration of the study Exclusion Criteria: * Chemotherapy or other investigational agent for the current episode of newly diagnosed atypical neurofibroma or MPNST * Prior therapy with an anti-PD-1, anti-PD-L1, or anti-PDL-2 antibody * Known allergy to compounds of similar chemical or biologic composition to Nivolumab or Ipilimumab * Pregnant or b…