Standard Treatment for Ependymoma
The standard treatment for ependymoma is maximal safe surgical resection followed by radiotherapy, with specific protocols determined by tumor grade, location, extent of resection, and patient age. 1
Surgical Management
- Gross total resection (GTR) is the cornerstone of treatment for all ependymomas, with level B evidence supporting this approach 1, 2
- Postoperative MRI should be performed to evaluate the extent of resection
- A second-look surgery should be considered when the first resection is incomplete and GTR is achievable 1
- Disease staging with both craniospinal MRI and CSF cytology is mandatory 2-3 weeks after surgery due to risk of CSF dissemination 1
Radiotherapy Guidelines
For Adults:
- WHO grade III (anaplastic) ependymomas: Postoperative conformal radiotherapy with doses up to 60 Gy regardless of extent of resection 1
- WHO grade II ependymomas: Postoperative conformal radiotherapy with doses of 54-59.4 Gy following incomplete resection 1
- Spinal ependymomas: Radiotherapy is reserved for incompletely resected tumors, typically with doses ≥50 Gy 2, 3
For Children:
- Children >18 months: Postoperative conformal radiotherapy with doses up to 59.4 Gy 1
- Children 12-18 months or older with poor neurological status: Radiotherapy with doses of 54 Gy 1
- Children <18 months: Chemotherapy alone is an option 1
- Children <12 months: Chemotherapy is recommended to delay radiation 1
Special Considerations
- In case of CSF or spinal dissemination, craniospinal irradiation (CSI) of 36 Gy is recommended with a boost up to 45-54 Gy on focal lesions 1
- For myxopapillary ependymoma, careful surgical technique is critical to avoid capsular violation, which strongly correlates with recurrence 2
- For completely resected supratentorial ependymomas (particularly classic type), observation without immediate radiotherapy may be considered 4
Chemotherapy Role
- Limited role in initial treatment of ependymomas
- Primary indications:
- No standard chemotherapy regimen has been established, though cisplatin-based regimens are most commonly used 5
Molecular Considerations
- Nine molecular subgroups of ependymal tumors have been identified across different anatomical compartments with distinct genetic profiles 1
- Chromosome 1q gain in infratentorial tumors is associated with worse outcomes (5-year EFS 47.4% vs 82.8% without 1q gain) 4
- Future treatment protocols may be stratified based on molecular profiling rather than traditional factors 1
Follow-up Recommendations
- Long-term follow-up with contrast-enhanced MRI due to risk of asymptomatic and late relapses 1
- For children who received radiotherapy, serial monitoring of cognitive and endocrine functions is recommended 1
Management of Recurrence
- Consider re-operation as first option, particularly for supratentorial tumors 6, 7
- Re-irradiation may be considered for focal recurrences 6
- Chemotherapy for recurrent disease when surgery or radiation is not feasible 3, 5
The evidence strongly supports an aggressive surgical approach followed by appropriate radiotherapy as the mainstay of ependymoma treatment, with specific protocols tailored to patient age, tumor location, and extent of resection.