We wanted to present a rare case of metastatic grade 2 spinal ependymoma with an atypical course at the time of diagnosis. Temozolomide plus capecitabine chemotherapy was started in May 2018 on a 30-year-old female patient with sacral ependymoma who had extensive lung metastases at the time of diagnosis. The patient remained in remission for approximately 29 months, and the current chemotherapy was continued until it progressed in November 2020. According to this case report, a combination of temozolomide and capecitabine may be the best treatment option for ependymoma patients.
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