Ependymomas.

Abstract

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.