Objective: The current study was conducted to evaluate the effects of low-dose craniospinal irradiation (CSI) combined with chemotherapy on non-metastatic embryonal tumors in the central nervous system (CNS), including medulloblastoma and supra-tentorial primitive neuroectodermal tumors (ST-PNET).
Methods: All patients were treated according to the following protocol. After surgery, the patients < or =5 years old received 18 Gy and the patients >5 years old received 24 Gy CSI. The dose to the primary tumor bed was 39.6-54 Gy. Chemotherapy consisted of ifosfamide, cisplatin and etoposide (ICE chemotherapy).
Results: Sixteen patients aged 0.5-20.4 (median 6.1) years were enrolled and followed for 11-165 (median 112) months. Both 5-year actuarial overall survival (OAS) and progression-free survival (PFS) were 81% (95% confidence interval (CI): 62-100%) for the 16 patients. Both 5-year OAS and PFS were 82% (CI: 59-100%) for the patients with medulloblastoma and 80% (CI: 45-100%) for the patients with ST-PNET. Both 5-year OAS and PFS were 75% for the eight patients < or =5 years old and 88% for the eight patients >5 years old. Both 5-year OAS and PFS were 100% for six average-risk patients (3 years or older, total resection and posterior fossa) and 70% for 10 poor-risk patients (others). The median total intellectual quotient at the last follow-up was 85 (ranging from 48 to 103) in 12 patients who were followed for 3-145 (median 49) months. Eight patients received hormone replacement therapy.
Conclusion: Low-dose CSI and ICE chemotherapy may have a role as a treatment option for a subset of patients with non-metastatic embryonal tumors in the CNS.