2017 Volume 54 Issue 3 Pages 236-240
There are only a few reports of pediatric extracranial malignant germ cell tumors (MGCTs) in Japan. Thus, we retrospectively analyzed 50 cases of MGCTs in a single institute to assess the outcome, optimal therapy and prognostic factors. Our data included those of 50 cases of extracranial MGCTs from 1983 to 2015, and the median age at diagnosis was 2.0 (0.03–16.4). The median follow-up period from diagnosis was 2199 (76–7922) days. As for histopathological findings, there were 7 cases of dysgerminoma, 34 cases of yolk sac tumor, 4 cases of embryonal carcinoma, and 5 cases of mixed germ cell tumors. Front line therapies were a wait-and-see approach after total resection for 7 cases, carboplatin-based chemotherapy for 35 cases, VAC therapy for 6 cases (VCR, CPA, Act-D), and other therapies for 2 cases. The 5-year overall survival (OS) rates were 100% for all stages except stage IV (n=41) and 53.6% (13.2–82.5) for stage IV (n=7). There was a statistically significant difference between the two groups (p=6.84E-9). Among those diagnosed with MGCT after total resection, 7 out of 33 were selected for the wait-and-see approach after obtaining written informed consent from a person with parental authority. Two out of the 7 patients relapsed, treated with chemotherapy, and recovered. Stage IV was a strong unfavorable prognostic factor. The wait-and-see approach can be one of the optimal choices for stage I patients and those who have undergone total resection.
