Ependymoma Author : Doctor Maura Massimino 1 Creation date : February 2004

Keywords Disease name Definition Diagnostic criteria and differential diagnosis Etiology Genetics Clinical description Prognosis Diagnostic methods Epidemiology Management Unresolved questions References Abstract Ependymoma accounts for 10% of all pediatric central nervous system tumors and originates in ventricular spaces or from residual intra-parenchymal ependymal cells. The most common location in children is infratentorial. Symptoms depend upon the location of the tumor and can vary from intracranial hypertension for tumors originating in posterior fossa, behavioural changes and pyramidal signs for supratentorial tumors, to dysaesthesia for spinal ones. Etiology is unknown. A cytogenetic alteration involving chromosome 22 is commonly reported. Many questions are still under debate: the optimal radiotherapy volumes, doses and techniques, the usefulness of chemotherapy, the prognostic impact of grading, patient's age, tumor site. Prognostic factors related to genetic and molecular pattern are under evaluation. Surgery remains the main treatment for ependymoma, but complete resectability can be difficult, especially when tumor occurs infratentorially. Surgery should be therefore performed in one or more operations, according to the patient's neurological conditions, to decrease surgical morbidity, thereby increasing chances to implement subsequent treatment. Chemotherapy regimens adopted so far may enable a further surgery by reducing volume or infiltrating aspects of residual tumor. Radiotherapy allows controlling ependymoma; new radiotherapy treatment techniques, such as 3-D conformal radiotherapy, may enable the delivery of high radiation doses to small tumor volumes, improving the therapeutic ratio.