- Originally used in children under 3 years: Chemotherapy was originally considered for children younger than 3 years to defer onset of radiation therapy (17).
- Limited role at present: Cisplastin has a high response rate but little effect on survival. It is not a favored treatment now because tumors that progress during chemotherapy do not respond well to subsequent radiation, and a combination of chemotherapy and radiation does not improve overall survival (18). However, its use may make residual tumor more amenable to second-stage resection.
Current standard adjuvant therapy
- Early (or immediate) postoperative radiation therapy improves outcome.
- Improves survival: Patients who underwent surgery plus postoperative radiation had better overall survival rates and progression-free survival rates compared to those who had surgery alone (19).
- Prophylactic craniospinal radiation is not beneficial.
Conformal radiation therapy
- Present standard of care, compared to previous conventional radiotherapy: Conformal radiation therapy offers the best local control, event-free survival, and overall survival rates. It can be safely used to treat children younger than 3 years (20, 21).
- Protective of surrounding tissue: The highest doses of radiation are limited to the primary site, and the dose to normal tissue is decreased. The radiation is focused on residual tumor volume with a 10-mm tumor margin.
- Historical approach: Several studies in the past (with small numbers of patients) advocated deferment of radiation if gross total resection had been achieved, or if the child was younger than 3 years (3, 4, 12).
- Risk requiring reoperation: However, 20–30% of the tumors recurred within 2 years and required repeat surgery and then radiation.
- Close radiological surveillance: Close radiological surveillance is required if radiation is deferred.
No role exists for immunotherapy at present.