GCT-07. Central nervous system germ cell tumors of children in Slovakia from 2000 to 2020

Abstract

Abstract INTRODUCTION: Central nervous system germ cell tumors (CNS GCTs) are very heterogenous group of tumors, which are divided into two main subgroups - germinomas and non- germinomatous germ cell tumors (NGGCTs). NGGCTs consist of embryonal carcinomas, yolk sac tumors, choriocarcinomas, and teratomas - mature, immature, or with malignant transformation, as well as mixed types. CNS GCTs are rare tumors, accounting approximately for 4-5% of all brain tumors in children, with a peak of incidence between the ages of 10 and 15 years, affecting predominantly boys. METHODS: Retrospective study of a cohort of 34 patients, age 0-18 years old, with CNS germ cell tumors diagnosed and treated in one of three Slovak pediatric oncology centers over a 21-year period from 01/2000 to 12/2020. Treatment was done according to the SIOP strategy. RESULTS: Out of 34 patients 15 patients were treated in the largest center, in Bratislava and 19 patients in the remaining 2 centers. Germinomas occurred in 16 (47.1%) and non-germinomatous germ cell tumors in 18 (52.9%) patients, 7 of whom had teratomas. In our cohort, 24 patients (70.6%) survived with an average follow-up age of 8.5 years. Twenty two patients remain in the 1st remission of the disease, 2 patients got through after disease recurrence. Ten patients (29.4%) died, 9 patients due to disease progression and 1 patient due to treatment complications. CONCLUSION: The overall survival of children with CNS GCT is most dependent on the histologic subtype of the tumor. Overall survival in children with germinomas reaches 90% and with NGGCTs around 60-70%, depending on the extent of the disease. In our cohort, we have achieved 70.6% 5 year overall survival of patients.