Preoperative and intraoperative perfusion magnetic resonance imaging in a RELA fusion-positive anaplastic ependymoma: A case report

BACKGROUND: Ependymomas are rare neuroepithelial tumors thought to arise from radial glial precursor cells lining the walls of the ventricles and central canal of the brain and spinal cord, respectively. Histopathological classification, according to World Health Organization criteria, has only recently defined the RELA-fusion positive ependymoma. These tumors may account for 70% of supratentorial ependymomas in children and represent an aggressive entity distinct from other ependymomas. CASE DESCRIPTION: Here we present the case of a patient with RELA-fusion positive ependymoma of the frontal lobe in whom we used preoperative and intraoperative magnetic resonance (MR) perfusion imaging. In this first demonstrated intraoperative evaluation of MR perfusion in ependymoma, increased peripheral perfusion of the lesion in a ring-like manner with a discrete cutoff around the surgical margin correlated with intraoperative findings of a clear border between the tumor and brain, as well as pathological findings of increased MIB index and hypercellularity—specifically within solid tumor components. An abnormal perfusion pattern also suggested an aggressive lesion, which was later confirmed on pathological analysis. In addition, intraoperative MR perfusion improved detection of tumor tissue in combination with traditional T1-weighted contrast-enhanced methods, which increased extent of resection. CONCLUSIONS: MR perfusion imaging may be a useful method for delineating tumor aggressiveness and borders, which can be prognostic.