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Abstract INTRODUCTION Here, we discuss the presentation, histology, therapy, and outcome of germ cell tumors in children. METHODS Treatment outcome and management was assessed for children diagnosed with germ cell tumors from 2007 to 2017 at Kagoshima University. RESULTS Twenty-six patients (20 boys, 6 girls) with a mean age of 11.5 ± 4.9 years were included in this study. Patient tumor types included: germinoma (n = 19); immature teratoma (n = 3); yolk sac tumor (n = 3); choriocarcinoma (n = 1); embryonal carcinoma (n = 1). The most common patient clinical features were headache and vomiting associated with hydrocephalus. The median follow-up period was 96.5 months. Tumor location was pineal (n=9), bifocal (n=6), suprasellar (n = 5), basal ganglia (n=2), frontal lobe (n=2), and cerebellum (n=2). Surgical procedures included stereotactic biopsy (n=13), endoscopic third ventriculostomy and biopsy (n=8), and tumor decompression (n=5). All patients with germ cell tumors underwent adjuvant chemotherapy and radiation therapy; patients with germinoma or immature teratoma were still alive, while patients with embryonal carcinoma, yolk sac tumor, or choriocarcinoma had poor prognosis with a median survival of 16 months. CONCLUSIONS Patients with germinoma had a relatively good prognosis, while patients with embryonal carcinoma, yolk sac tumor, or choriocarcinoma had a poor prognosis. A multidisciplinary approach including surgical strategy based on location, appropriate radiation planning, and chemotherapy is needed for effective treatment and improved outcomes.