Ependymoma accounts for 1.2%–7.8% of intracranial tumors, with nearly 70% occurring in children. Approximately 60%–70% of these tumors are located in the infratentorial region. Ependymomas often originate in the fourth ventricle and may invade the foramen of Magendie and Luschka. These tumors typically appear gray in color with seemingly defined borders. Although they are less malignant compared to medulloblastomas, they have the potential for dissemination through cerebrospinal fluid (CSF), which is associated with a poor prognosis.
Patients frequently present with symptoms of increased intracranial pressure, vertigo, and ataxia. Tumors originating from the floor of the fourth ventricle are often associated with hydrocephalus. Ependymomas characterized by RELA fusion-positive genetic alterations represent a distinct molecular subtype, which accounts for approximately 70% of supratentorial ependymomas in children and is indicative of a poor prognosis.