Abstract

PurposeA growing number of young patients with central nervous system (CNS) tumour survived for more than five years. However, these long-term survivors might be at risk of multiple late effects thus leading to a higher risk of late mortality. We aimed to explore the risk of late mortality and the pattern of mortality among long-term survivors of childhood or adolescent CNS tumour.MethodsWe identified 5-year survivors with childhood or adolescent CNS tumour before age 20 years through the Swedish Cancer Registry. Five controls were randomly matched for each patient to generate the reference group. We retrieved information about death via Cause of Death Register. We calculated the absolute excess risk (AER) of death and the hazard ratio (HR) of death using Cox proportional hazard model.ResultsLong-term survivors with CNS tumour suffered a significant higher risk of overall mortality (HR 6.56, 95% CI 5.71–7.53; AER 5.89, 95% CI 5.03–6.87). The mortality rate declined with the increasing survival time, but it was still higher even after 30 years of follow-up. Malignant neoplasms contributed mostly to late mortality with an AER of 3.75 (95% CI 2.95–4.75). Female survivors, survivors diagnosed at a younger age and survivors with medulloblastoma were particularly strongly associated with a higher risk of death.ConclusionsLong-term survivors of childhood and adolescent CNS tumours are at a higher risk of late mortality, and the risk of death is affected by gender, age at diagnosis and types of CNS tumour.

Highlights

  • Central nervous system (CNS) tumour was the first most commonly newly-diagnosed type of tumour in Swedish population before age 20 years in 2016, with age-standardised incidence 4.7 per 100,000 person-years in girls and 3.7 in boys [1]

  • Journal of Neuro-Oncology (2019) 145:541–549 the overall and cause-specific mortality among long-term survivors with childhood cancers [3,4,5,6,7,8,9,10], whereas limited evidence existed on investigating the patterns of death cause among survivors with CNS tumour diagnosed in children or adolescents

  • In this population-based cohort study utilising a range of Swedish nationwide registers, we aimed to explore the mortality rate and the mortality patterns among long-term survivors who were diagnosed with CNS tumour before 20 years of age

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Summary

Introduction

Central nervous system (CNS) tumour was the first most commonly newly-diagnosed type of tumour in Swedish population before age 20 years in 2016, with age-standardised incidence 4.7 per 100,000 person-years in girls and 3.7 in boys [1]. Journal of Neuro-Oncology (2019) 145:541–549 the overall and cause-specific mortality among long-term survivors with childhood cancers [3,4,5,6,7,8,9,10], whereas limited evidence existed on investigating the patterns of death cause among survivors with CNS tumour diagnosed in children or adolescents. In this population-based cohort study utilising a range of Swedish nationwide registers, we aimed to explore the mortality rate and the mortality patterns among long-term survivors who were diagnosed with CNS tumour before 20 years of age. We investigated whether the mortality patterns were associated with survival time and with specific types of CNS tumour

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