Medical Oncology Unit, San Raffaele Scientific Institute, Milan, Italy. reni.michele@hsr.it
Ependymomas
are rare tumours of neuroectodermal origin classified as myxopapillary
ependymoma and subependymoma (grade I), ependymoma (grade II) and
anaplastic ependymoma (grade III). The more common location is
infratentorial (60%). Age <40 years and extent of surgery appear
related to better prognosis, while the role of other prognostic
factors, such as tumour grade and tumour site are equivocal. This
emphasizes the role of surgery as the standard treatment.
Postoperative
radiotherapy is indicated in high-grade ependymomas, and is recommended
in low-grade ependymomas after subtotal or incomplete resection
(confirmed by postoperative MR). Deferral of radiotherapy until
recurrence may be considered on an individual basis for patients with
MR confirmation of a radical resection. Recommended dose to involved
fields is 45-54 Gy for low-grade (grade II) and 54-60 Gy for high-grade
ependymomas (grade III). There is no proof that postoperative
chemotherapy improves the outcome. At recurrence, platinum-,
nitrosourea- or temozolomide-based chemotherapy can be administered,
although there is no evidence of efficacy.