Intracranial ependymoma in pediatric patients. A review of six cases.

Intracranial ependymoma is an infrequent tumor in children, accounting for less than 15% of all brain tumors and less than 5% of all cancers seen in the pediatric population. Three of every four cases develop under the tentorium. A small number of cases confined to the spine have been reported in children. Nearly 50% of patients are younger than four years at diagnosis. Inaugural symptoms are usually abrupt in onset and lacking in specificity, being dominated by vomiting and behavioral disorders. Older children tend to present with more specific manifestations such as headache, seizures, motor disorders, and cerebellar dysfunction. The diagnosis rests on imaging studies and on histologic examination of the operative specimen. Some patients require placement of a ventricular shunt prior to surgical removal of the tumor. The prognosis remains poor, with a five-year survival rate of 16% to 67%. Although few predictive factors are agreed on, a poor prognosis seems more common in very young patients, when excision is incomplete, when recurrences develop, and/or when the tumor site is not treated by radiation. The authors' experience with six children is reported.