Anaplastic Ependymoma and Posterior Fossa Grouping in a Patient with H3K27ME3 Loss of Expression but Chromosomal Imbalance

Ependymoma represents the third most common childhood malignancy of the brain, accounting for approximately 10% of all pediatric brain malignancies.1 The most common site of presentation is the posterior fossa.1 Standard therapy involves maximal safe resection, followed by radiation therapy (RT).2 The benefit of chemotherapy (CHT) has not been demonstrated and remains under investigation.3,4 Molecular analysis has identified 2 groups within ependymomas of the posterior fossa (PF-EPN) with distinct prognoses and characteristics (Table 1): group A (PF-EPN-A) and group B (PF-EPN-B).