Friederike Erdmann

Background Tumors of the central nervous system (CNS) are the second most common type of pediatric cancer in Germany. We aimed to describe registration practice, incidence, and survival patterns for childhood CNS tumors in Germany for the past 40 years. Procedure Including all CNS tumor cases in children diagnosed at ages 0–14 years registered at the German Childhood Cancer Registry (GCCR) in 1980–2019 (for survival analysis 1980–2016), we calculated age‐specific and age‐standardized incidence rates (ASIR) over time, average annual percentage changes (AAPC), and 1‐ and 5‐year overall survival. Results While we observed a pronounced increase in ASIR after the establishment of the GCCR during the 1980s, ASIR for all pediatric CNS tumors combined continued to increase markedly from 28.6 per million in 1990–1999 to 43.3 in 2010–2019 (AAPC = 2.7% in 1991–2010, AAPC = 0.3% in 2010–2019). The 5‐year overall survival from CNS tumors improved from 63% in the 1980s, 70% in the 1990s to 79% in 2010–2016. These improvements have occurred across all age groups. Children diagnosed with ependymomas and choroid plexus tumors experienced the strongest increase (from 54% to 81%). Conclusions Observed increases in incidence rates for pediatric CNS tumors are likely only partially caused by actual increasing case numbers. The majority is a function of improved registration and, to a minor extent, improvements in diagnostics. Survival from pediatric CNS tumors has, by and large, improved consistently, leading to a growing population of childhood cancer survivors with diverse health biographies and risk of lifelong adverse impact on health and wellbeing.