radical resection, including the tumor infiltrating the brainstem. While this approach offers a significantly greater chance of long-term survival, it also significantly increases the risks of serious and permanent neurologic disability; the risks of such an aggressive approach must be thoroughly discussed with the family.

Although several small series have suggested that some patients with ependymoma treated only by gross tumor resection are long-term survivors [22], most clinicians have followed surgical resection with radiation therapy. Several single-institution studies have shown durable progression-free survival in greater than 50% of patients receiving focal radiotherapy following gross total resection, while children with significant post-surgery residual tumor have considerably worse survival, even with radiotherapy [23].

While several chemotherapeutic agents have demonstrated activity in ependymoma, no randomized trials have shown that the addition of chemotherapy to post-operative radiotherapy improves prognosis. When an aggressive chemotherapeutic protocol was used without radiation therapy in the treatment of infants with ependymoma, the 5-year progressionfree survival was 34% [24]. Thus, the role of chemotherapy in the treatment of ependymoma has yet to be defined. A study soon to open in the COG will evaluate the use of chemotherapy in patients with incompletely resected tumors to render these tumors resectable at second-look surgery.

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