Ependymoma

Abstract Ependymomas are uncommon glial neoplasms whose biology and clinical characteristics are poorly understood and for which there are few effective treatment options. They occur predominantly in the pediatric as well as the adolescent and young-adult age groups and are seen mainly in the posterior fossa and spinal cord. They range in behavior from low-grade slow-growing tumors such as myxopapillary ependymomas to aggressive malignancies such as anaplastic ependymomas. Intense research in recent years has yielded novel insights into the biology and clinical behavior of these tumors, which has allowed development of several targeted therapeutic approaches. However, the modest efficacy of such studies to date have emphasized the complexity of these tumors and highlighted the need for continued preclinical and translational research to address their clinical impact. This chapter reviews the current state of the science of biology, pathophysiology, and classification of ependymomas and outlines the current recommendations for treatment of patients with these tumors.