DDDR-34. RECURRENT RELA FUSION-POSITIVE EPENDYMOMA TREATED WITH VAL-083 UNDER EXPANDED ACCESS: A CASE REPORT

Abstract Ependymoma is a relatively rare central nervous system tumor accounting for 2-9 % of all neuroepithelial tumors. RELA fusion-positive ependymoma subgroup associated with supratentorial location, higher WHO grade and worse prognosis. In addition, there no standard systemic chemotherapy treatment adults recurrent disease. VAL-083 bi-functional DNA-targeting agent which rapidly induces inter-strand DNA cross-links at N7-guanine inducing double-strand breaks causing cell death acts independent MGMT DNA-repair in high-grade gliomas. We report 40-year-old male who was initially diagnosed right parieto-occipital glioma, somatic mutations including IDH1/2 genes, unmethylated promoter status. He underwent gross total resection, followed by chemoradiation concurrent adjuvant temozolomide 12 cycles. Sixteen months later, he developed disease had second resection. Inter- intragenic fusion analysis tissue revealed C11orf95-RELA the diagnosis established. The patient not eligible to participate any clinical trial received under an expanded access program. then treated (30 mg/m2 3 consecutive days every 21 days) completed cycles during period 9 months. No 3/4 adverse events such as thrombocytopenia, anemia, neutropenia, or lymphopenia were observed. His liver renal functions normal. dose reduction required. also levetiracetam, alprazolam prochlorperazine, drug interactions. Steroid has been surveillance since completion remained radiologically stable, CSF dissemination, after This case highlights that may be option refractory temozolomide-based regimens. Clinicaltrials.gov Identifier: NCT03138629. plans this EAP approved MD Anderson Cancer Center IRB.