Treatment is multidisciplinary:
- Radiation therapy (RT)
Surgery for these tumors is rarely curative alone.
Complete macroscopic resection is possible in:
- >80% supratentorial and spinal tumors
- <50% posterior fossa tumors
Posterior fossa ependymomas are usually attached to the floor of the 4th ventricle, and complete resection is difficult to achieve.
Gross total resection of tumor in any location improves local control rates and - if surgery is not going to be too damaging - should always be aimed for.
Patients with complete resection and radiation therapy have a 5-year survival estimate of about 65 to 80%. In the case of incomplete resection the 5 year survival is just under 50%.
In the previous completed COG trial (ACNS0121):
- Patients who have had a gross total resection of a low grade supratentorial ependymoma were observed only and given no adjuvant therapy.
- If the tumor is unresectable/subtotally resected initially patients were treated with 7 weeks of chemotherapy (Carboplatin, Vincristine, Cyclophosphamide, Etoposide) and then the possibility of second look surgery with re-resection was recommended.
For very young children (age < 3 years), post-operative radiotherapy (RT) is generally felt to be associated with too much toxicity and so chemotherapy has been tried to delay the start of any RT.
The Head Start Protocols using high dose chemotherapy alone after surgery have had very disappointing results for ependymoma.
Recent evidence has emerged that chemotherapy may reduce the risk of local relapse, but does not significantly affect survival rates.
ACNS0121 used chemotherapy after subtotal resection with Vincristine, Carboplatin, and cyclophosphamide alternating with Vincristine, Etoposide, and Cisplatin. Second look surgery with re-resection was then recommended prior to local RT.
However chemotherapy is not very effective in this disease and can never salvage a child with relapse.