Abstract

Abstract BACKGROUND Phase II clinical trial funded by Ministry of Health, Labour and Welfare from 1995 to 2003 evaluated efficacy of pathology-based three-group treatment stratification for CNS germ cell tumors (GCTs). We here present long-term follow-up results. METHODS Total 228 cases were registered. Germinoma was treated with carboplatin+etoposide (CARE) and extended-local irradiation, local irradiation was added for intermediate-prognosis-group, and poor-prognosis-group was treated with ifosfamide+cisplatin+etoposide (ICE) and whole-brain or craniospinal irradiation. RESULTS Mean/median ages at diagnosis were 16.8/16 years and female-to-male ratio was 40-188. Registry included 124 germinomas, 75 intermediate-prognosis-group cases (including 37 germinomas with STGC), 28 poor-prognosis-group cases and 1 mature teratoma. Median 222-months follow-up was conducted, and 56 recurrences and 39 deaths were recorded. 10 and 20-year event-free survival (EFS) for germinoma, intermediate and poor-prognosis-groups were 82/73%, 76/66% and 49/49%, respectively, and overall survival (OS) for each were 97/92%, 87/70% and 61/53%, respectively. Prognosis for germinoma with or without STGC was not statistically different. Basal ganglia germinoma showed significantly shorter RFS but OS was not different from other locations. Median age at death was 24 years, Time from treatment to deaths was significantly shorter for deaths due to original disease (5.7 years) than complications (14.9 years) and other causes (12.7 years) (p=0.005, 0.04, respectively). Overall survival after recurrence was clearly distinct according to the original histological groups; overall survival for germinoma, intermediate prognosis group and poor prognosis group were 94, 88 and 18% at 1 year, 91, 50 and 9% at 2 years and 88, 38 and 9 % at 3 years, respectively. CONCLUSIONS Germinoma and intermediate-prognosis-group cases showed long-term survival for approximately 90%, while more intensive treatment would be necessitated for poor-prognosis-group. Long-term survivors often required hormonal supplementation, and increasing frequency of treatment-related complications was observed. There is no end of outpatient follow-up for CNS GCT patients.

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