Ependymoma Guidelines

Updated: Mar 09, 2023
  • Author: Jeffrey N Bruce, MD; Chief Editor: Herbert H Engelhard, III, MD, PhD, FACS, FAANS  more...
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Guidelines Summary

The National Comprehensive Cancer Network (NCCN) recommends the following for treatment of adults with ependymoma [3] :

  • Following suspicion for ependymoma on neuroimaging, maximal safe resection should be performed
  • Following diagnosis of grade 2 or grade 3 ependymoma after resection/biopsy, conduct brain and spine MRI, plus LP to assess for leptomeningeal spread (LP contraindicated with posterior fossa masses) 
  • If post-resection neuroimaging is negative for metastasis, administer standard conformal radiation therapy  (to tumor area plus 1-2cm margins). If metastasis detected, administer whole craniospinal radiation therapy (or proton therapy to reduce toxicity.) Spinal ependymomas < WHO Grade 2 do not require radiation therapy if gross total resection was performed and neuroimaging/LP are negative.
  • MRI surveillance for recurrence following resection for 5-10 years
  • For recurrent ependymoma, patients who have not received radiation therapy should receive radiation therapy, and if a patient has received radiation therapy, then chemotherapy, radiation therapy, or supportive care should be considered.

The European Association of Neuro-oncology (EANO) recommends a similar treatment approach to the NCCN, advocating for maximal safe resection followed by post operative radiation and monitoring for recurrence. However, the EANO recommends adjuvant chemotherapy (exact regimen varies) instead of radiation in patients less than 12 months old to avoid harmful side effects of radiotherapy. On the contrary, in the NCCN guidelines, chemotherapy is not recommended as a monotherapy and is only considered in recurrent cases where resection and radiation have failed. [68]