The legacy nomenclature of RELA fusion-positive ependymomas that appeared in WHO CNS 4 has been updated based on evidence that the fusion partner gene ZFTA (previously called C11ORF95) is more recurrent in this entity and can be rearranged with partners other than RELA. ZFTA fusion ependymomas are more common than YAP1 and typically occur in the frontal and parietal lobes, with a median age of approximately 6.5 years at the time of surgery [33, 34]. The gene discussed is YAP1; the disease is ependymoma.