Optimal management of
ependymomas includes surgical resection and evaluation of the extent of central nervous system involvement using cerebrospinal fluid cytology and craniospinal contrast-enhanced MRI. In instances of measurable residual disease, reoperation should be considered because survival of patients with
ependymomas is significantly improved by performance of a complete resection. In patients not considered for further surgery and with residual disease, limited-field
radiotherapy is usually administered. The role of
craniospinal irradiation in patients with local disease and no evidence of
metastasis is controversial because most
tumor recurrences are local and at the site of the primary
tumor. No clear role for
adjuvant chemotherapy has been demonstrated. When used,
chemotherapy for
ependymomas has been administered primarily to children aged younger than 3 years as adjuvant
therapy and to patients with recurrent disease who are not considered surgical candidates as
salvage therapy. Recurrent
ependymomas are managed by reoperation of
tumors that are surgically accessible, by
radiotherapy if not previously administered, and by salvage
chemotherapy. The role of stereotactic
radiotherapy administered as radiosurgery or
brachytherapy is unclear because all reports are anecdotal. Because salvage
chemotherapy is not curative, no standard
therapy exists, and a variety of
chemotherapy agents and
drug schedules have been investigated.