EPEN-06. CHARACTERISING THE DEVELOPMENTAL ORIGINS AND TRAJECTORIES OF ZFTA-RELA FUSION EPENDYMOMA IN A NOVEL MOUSE MODEL

Abstract Ependymomas driven by the ZFTA-RELA fusion account for >70% of all supratentorial ependymomas. These tumours are now recognised in WHO classification CNS and have been associated with a poor prognosis. Seven variants described: around two thirds cases 1. No spontaneous genetically modified mouse models (GEMMS) described current require invasive intracranial injection (of transduced cells or RCAS-TVA system). Here we describe first GEMM fusion-driven ependymoma. Nestin-Flx-STOP-Flx-ZFTA-RELA (Fusion 1) open reading frames were targeted together luciferase to Rosa-26 locus. Breeding these mice Nestin-CreERT2 Blbp-Cre lines that drive recombination neural progenitor resulted forebrain could be tracked bioluminescence imaging from P20. Tumours displayed NF-kB L1CAM expression protein was detected using novel in-house antibody. display known ependymoma transcriptomic signature. can grown as neurospheres passaged allografts nude respond irradiation. We provide this important group present our histologic developmental timecourse tumours, their comparison human disease further understanding lineage origins potential targets.