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Surgery
Source: Childhood Ependymoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: January 6, 2025 · Captured 2026-09-09.
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Context: Treatment of Childhood Intracranial Ependymoma
Surgery is performed in an attempt at maximal tumor reduction. Evidence suggests that more extensive surgical resection is related to an improved rate of survival.[1-5]; [6,7][Level of evidence C2] Magnetic resonance imaging (MRI) is performed postoperatively to confirm the extent of resection. If not obtained preoperatively, MRI of the entire neuraxis and cerebrospinal fluid cytopathology is performed to evaluate for disease dissemination.
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Patients across all molecular subgroups who have residual tumor or disseminated disease are considered at high risk of relapse and may be treated on clinical trials specifically designed for them. Patients with no evidence of residual tumor still have an approximate 20% to 40% relapse risk despite receiving postoperative radiation therapy.[8][Level of evidence B4]
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Anecdotal experience suggests that surgery alone for completely resected supratentorial World Health Organization (WHO) grade 2 tumors and spinal ependymomas may, in select cases, be an appropriate approach to treatment.[9-13][Level of evidence C2]
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Evidence (surgery):
A prospective multi-institutional cooperative group trial (Children's Oncology Group [COG] ACNS0121 [NCT00027846]) included patients with newly diagnosed intracranial ependymomas (N = 356). Surgery alone was used for the treatment of supratentorial, WHO grade 2, gross-totally resected ependymomas (n = 11).[8][Level of evidence B4]
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A prospective multi-institutional cooperative group trial (Children's Oncology Group [COG] ACNS0121 [NCT00027846]) included patients with newly diagnosed intracranial ependymomas (N = 356). Surgery alone was used for the treatment of supratentorial, WHO grade 2, gross-totally resected ependymomas (n = 11).[8][Level of evidence B4]
The 5-year event-free survival (EFS) rate was 61.4%, and the overall survival (OS) rate was 100%.
A prospective multi-institutional cooperative group trial (Children's Oncology Group [COG] ACNS0121 [NCT00027846]) included patients with newly diagnosed intracranial ependymomas (N = 356). Surgery alone was used for the treatment of supratentorial, WHO grade 2, gross-totally resected ependymomas (n = 11).[8][Level of evidence B4]
Local failure occurred in four patients (36%), and local and distant failure occurred in one patient (9%).
A prospective multi-institutional cooperative group trial (Children's Oncology Group [COG] ACNS0121 [NCT00027846]) included patients with newly diagnosed intracranial ependymomas (N = 356). Surgery alone was used for the treatment of supratentorial, WHO grade 2, gross-totally resected ependymomas (n = 11).[8][Level of evidence B4]
In this study, the number of patients eligible for a surgery-alone approach was very small. Only a subset of these patients successfully avoided additional treatment.
Retrospective analysis of the outcome for patients with posterior fossa B ependymoma suggests that these patients might be sufficiently treated with gross-total resection alone,[7] but this approach has not been tested in a prospective randomized clinical trial.
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