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. 2022 Apr;157(2):307-317.
doi: 10.1007/s11060-022-03963-3. Epub 2022 Feb 11.

Central nervous system tumors in children under 5 years of age: a report on treatment burden, survival and long-term outcomes

Sarah Metzger  1 , Annette Weiser  1 , Nicolas U Gerber  1 , Maria Otth  1   2 , Katrin Scheinemann  2   3   4 , Niklaus Krayenbühl  5 , Michael A Grotzer  1 , Ana S Guerreiro Stucklin  6
Affiliations

Central nervous system tumors in children under 5 years of age: a report on treatment burden, survival and long-term outcomes

Sarah Metzger et al. J Neurooncol. 2022 Apr.

Erratum in

Abstract

Purpose: The challenges of treating central nervous system (CNS) tumors in young children are many. These include age-specific tumor characteristics, limited treatment options, and susceptibility of the developing CNS to cytotoxic therapy. The aim of this study was to analyze the long-term survival, health-related, and educational/occupational outcomes of this vulnerable patient population.

Methods: Retrospective study of 128 children diagnosed with a CNS tumor under 5 years of age at a single center in Switzerland between 1990 and 2019.

Results: Median age at diagnosis was 1.81 years [IQR, 0.98-3.17]. Median follow-up time of surviving patients was 8.39 years [range, 0.74-23.65]. The main tumor subtypes were pediatric low-grade glioma (36%), pediatric high-grade glioma (11%), ependymoma (16%), medulloblastoma (11%), other embryonal tumors (7%), germ cell tumors (3%), choroid plexus tumors (6%), and others (9%). The 5-year overall survival (OS) was 78.8% (95% CI, 71.8-86.4%) for the whole cohort. Eighty-seven percent of survivors > 5 years had any tumor- or treatment-related sequelae with 61% neurological complications, 30% endocrine sequelae, 17% hearing impairment, and 56% visual impairment at last follow-up. Most patients (72%) attended regular school or worked in a skilled job at last follow-up.

Conclusion: Young children diagnosed with a CNS tumor experience a range of complications after treatment, many of which are long-lasting and potentially debilitating. Our findings highlight the vulnerabilities of this population, the need for long-term support and strategies for rehabilitation, specifically tailored for young children.

Keywords: Brain tumors; Educational/occupational outcomes; Long-term outcome; Pediatrics; Survival.

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Conflict of interest statement

The authors have no relevant financial or non-financial interests to disclose.

Figures

Fig. 1
Fig. 1
Characteristics of Study Population. A Tumor subgroups in percentage of whole cohort. B Age distribution according to tumor type. C Location of tumors according to tumor type. D Progression free survival and overall survival of whole cohort with 95%-confidence intervals and corresponding risk table. Censored cases are depicted as crosses
Fig. 2
Fig. 2
Kaplan–Meier Curves A–D depicting progression free survival and overall survival of the four main tumor subgroups with corresponding risk-tables. Censored cases are depicted as crosses
Fig. 3
Fig. 3
Educational/occupational outcomes by age at last follow-up
See this image and copyright information in PMC

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