Ependymoma Treatment Guidelines

Introduction to Ependymoma Treatment

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, as recommended by Neuro-Oncology guidelines 1

Gross total resection (GTR) is the cornerstone of treatment for all ependymomas, with level B evidence supporting this approach, according to Neuro-Oncology guidelines 1
A second-look surgery should be considered when the first resection is incomplete and GTR is achievable, as suggested by Neuro-Oncology guidelines 1
Postoperative MRI should be performed to evaluate the extent of resection, and disease staging with both craniospinal MRI and CSF cytology is mandatory 2-3 weeks after surgery due to risk of CSF dissemination, as recommended by Neuro-Oncology guidelines 1
Careful surgical technique is critical to avoid capsular violation, which strongly correlates with recurrence, according to Neuro-Oncology guidelines 1

For WHO grade III (anaplastic) ependymomas, postoperative conformal radiotherapy with doses up to 60 Gy is recommended, regardless of extent of resection, according to Neuro-Oncology guidelines 1
For WHO grade II ependymomas, postoperative conformal radiotherapy with doses of 54-59.4 Gy is recommended following incomplete resection, as suggested by Neuro-Oncology guidelines 1
For children >18 months, postoperative conformal radiotherapy with doses up to 59.4 Gy is recommended, according to Neuro-Oncology guidelines 1
For children 12-18 months or older with poor neurological status, radiotherapy with doses of 54 Gy is recommended, as suggested by Neuro-Oncology guidelines 1
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, according to Neuro-Oncology guidelines 1
Postoperative radiotherapy with doses ≥50 Gy is recommended only in cases of incomplete resection (Level B evidence), and for patients with GTR, observation without radiotherapy is appropriate, according to Neuro-Oncology guidelines 1
Radiotherapy increases local control and progression-free survival, with 10-year progression-free survival improving from <40% to 70%, and delaying or omitting radiotherapy after incomplete resection may lead to progression and poorer outcomes, as reported by Neuro-Oncology guidelines 1

Chemotherapy alone is an option for children <18 months, and is recommended to delay radiation for children <12 months, as suggested by Neuro-Oncology guidelines 1
Primary indications for chemotherapy include young children <12 months (to delay radiation) and recurrent disease when further surgery and radiation are not feasible, according to Neuro-Oncology guidelines 1

Nine molecular subgroups of ependymal tumors have been identified across different anatomical compartments with distinct genetic profiles, as reported by Neuro-Oncology guidelines 1
Future treatment protocols may be stratified based on molecular profiling rather than traditional factors, according to Neuro-Oncology guidelines 1
Long-term follow-up with contrast-enhanced MRI is recommended due to risk of asymptomatic and late relapses, and serial monitoring of cognitive and endocrine functions is recommended for children who received radiotherapy, as suggested by Neuro-Oncology guidelines 1
Overall survival at 10 years exceeds 90%, according to Neuro-Oncology guidelines 1
Presacral MPE shows worse outcomes compared to MPE of the filum terminale/cauda equina region, and clinical presentation depends primarily on tumor location and size, typically with symptoms related to spinal cord compression, as reported by Neuro-Oncology guidelines 1

In case of relapse, consider re-operation, re-irradiation, or chemotherapy, and comprehensive pituitary function testing should be performed for patients with MPE who develop endocrine abnormalities, as suggested by Neuro-Oncology and EANO guidelines 1
When planning treatment for MPE located near the pituitary region, the potential risk to endocrine function should be considered in the treatment planning, and serial monitoring of cognitive and endocrine functions following radiotherapy is recommended, particularly when considering potential pituitary effects, as recommended by EANO guidelines 1