Ependymoma

Ependymomas represent the third most common central nervous system tumours in childhood, arising anywhere across the entire neuraxis, albeit most frequently in the posterior fossa. Once considered single entities, these neuroepithelial malignant tumours are now known to demonstrate marked heterogeneity in clinical, pathological, and molecular characteristics with consequence on treatment responsiveness and patient prognosis. Indeed, finding curative therapy for certain ependymoma subtypes remains one of the biggest challenges in paediatric neuro-oncology. This chapter, with a focus towards paediatric intracranial ependymomas, will summarise the current state-of-play regarding our understanding of biology, current therapeutic strategies, and international clinical trials, with considerations for future progress in the field.