Low-dose Craniospinal Irradiation and Ifosfamide, Cisplatin and Etoposide for Non-metastatic Embryonal Tumors in the Central Nervous System

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 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 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. Key words: medulloblastoma – primitive neuroectodermal tumor – chemotherapy – radiotherapy – late effec