Cerebral ependymoma
ICD-10 C71.9 · ICD-11 2A00.0Y&XH1511&XA9738

Treatment of Newly Diagnosed Cerebral Ependymoma in Children Older Than 18 Months When Surgical Resection Did Not Achieve Complete Removal

Clinical scenario

This protocol addresses children older than 18 months with a newly diagnosed, localized intracranial ependymoma (WHO grade II or III), with no evidence of CSF or spinal dissemination.

When the prior step was not sufficient

The first step in this setting is surgical resection, with the goal of gross total removal confirmed by no residual tumor on postoperative MRI. When that endpoint is not reached — when residual disease remains visible — this protocol defines the subsequent management.

Treatment approach (partial overview)

Management involves postoperative conformal radiotherapy directed to the tumor bed, with chemotherapy delivered as an adjunct. The complete regimen — including sequencing, additional measures for visible residual disease, and the full set of options — is detailed in the structured protocol below.

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References

DOI: 10.1093/neuonc/nox166

  1. Key recommendations for the treatment of newly diagnosed intracranial WHO grades II and III ependymomas in children.
  2. Postoperative conformal radiotherapy with doses up to 59.4 Gy is recommended in children older than 18 months.
  3. Postoperative chemotherapy, using various combinations of etoposide, vincristine, cyclophosphamide, platinum derivatives, and high-dose methotrexate, showed a 40% to 50% response rate.
  4. As there is reluctance to deliver radiation to very young children, postoperative chemotherapy has been frequently proposed, while in older children chemotherapy is delivered as an adjunct to radiotherapy.
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