Management of Ependymoma in Children, Adolescents and Young Adults.

ABSTRACT

Paediatric ependymomas are rare, malignant tumours arising throughout the central nervous system, but most frequently (in children) the posterior fossa. The standard of care for localised disease is gross total resection and focal radiotherapy, resulting in overall survival rates of up to 85%. Despite improvements in survival, treatment remains challenging, with persistently high rates of (rarely curable) relapse alongside risks of significant tumour and treatment-related toxicity. Systemic therapy is currently used to delay radiotherapy in very young children and in the management of metastatic or recurrent disease. Its use in the adjuvant setting is the subject of ongoing studies. Current research efforts are aimed at eliciting a better understanding of molecular biology, correlating this with tumour behaviour and defining targets for potential new agents. Prognosis seems to be related to the extent of surgical resection and the age at presentation. This article reviews clinical aspects of ependymoma management in children and young people.