ORIGINAL PAPER Pediatric central nervous system tumors: review of a single Portuguese institution Maria M. Santos 1 & Cláudia C. Faria 1 & José Miguéns 1 Received: 28 February 2016 /Accepted: 13 April 2016 # Springer-Verlag Berlin Heidelberg 2016 Abstract Introduction Despite being the second most frequent tumor in children, pediatric central nervous system (CNS) tumors are rare, and the published European epidemiological data is limited. Our goal is to present the first surgical series of pediatric CNS tumors in Portugal and to review other similar worldwide series. Methods Retrospective review of all patients younger than 19 years old, operated to a CNS tumor in the Neurosurgery Department at Hospital de Santa Maria (Lisbon, Portugal) between January 2004 and December 2014. Demographic data, tumor location, clinical data, histopathology, and surgi- cal treatment were analyzed and compared to surgical series of pediatric CNS tumors published in PubMed indexed journals over the last 20 years. Results We performed 253 surgeries in 215 patients, with a male:female ratio of 1.2:1 and a mean age of 9.2 years old. Primary brain tumors accounted for 95 % of all tumors and had more often a supratentorial location. Tumors of neuroepithelial tissue, particularly astrocytic tumors, embryo- nal tumors, neuronal and mixed neuronal-glial tumors, and oligodendrogliomas accounted for 81 % of cases. A gross- total resection was achieved in most cases. There was no mor- tality, and the overall morbidity was low. Conclusions The demography, topography, and clinical pre- sentation of the tumors and the surgical results of this series are comparable to other European ones. We found a higher incidence of neuronal and mixed neuronal-glial tumors and oligodendrogliomas and a slight lower incidence of ependymomas. Our results should encourage further national multi-institutional studies to better characterize these tumors in the pediatric population. Keywords Central nervous system . Pediatric tumors . Epidemiology . WHO classification Introduction Central nervous system (CNS) tumors are the most common solid tumors of childhood, accounting for more than 20 % of all pediatric cancers [1]. Given their particular topography and biological behavior, they are usually analyzed separately from adult CNS tumors. The annual incidence of pediatric brain tumors is estimated to be approximately 2.9 and 5.05/100,000 children in Europe and in the United States (US), respectively [2–6]. In Northern Europe, there is an apparent greater inci- dence of these tumors when compared to southern countries [5] but this fact may be due to a higher notification rate [7]. Portugal, a southern European country with 10.5 million inhabitants, has less than 1.6 million citizens aged 18 or youn- ger. To our knowledge, the epidemiological data of CNS tu- mors in Portugal has not yet been published. We have ana- lyzed a series of CNS tumors in patients under 19 years of age treated at our institution, Hospital de Santa Maria (Lisbon, Portugal). Furthermore, we did compare our results to those reported in other countries. Methods A retrospective review of all operations for pediatric CNS tumors performed in the Neurosurgery Department at * Maria M. Santos mariamanesantos@gmail.com 1 Department of Neurosurgery, Hospital de Santa Maria-Centro Hospitalar Lisboa Norte, E.P.E., Av. Professor Egas Moniz, 1649-035 Lisbon, Portugal Childs Nerv Syst DOI 10.1007/s00381-016-3088-0