The role of sex genotype in paediatric CNS tumour incidence and survival

Wai Cheong Soon,Sandeep Solanki,Josie Hayes,Susan Picton,Edward Goacher,Paul Dominic Chumas,Melpo Kapetanstrataki,Ryan Koshy Mathew

doi:10.1007/s00381-021-05165-0

Abstract

PurposeEvidence exists, in CNS germinomas and medulloblastomas (MB), that patient sex significantly influences incidence and outcome. The role of sex genotype in other paediatric CNS tumours remains unclear. This study sought to examine the role of sex genotype in CNS tumour incidence and overall survival (OS).MethodsAge-adjusted incidence and OS rates were collected from the Surveillance Epidemiology and End Result (SEER) registry between 2000 and 2011 for common paediatric (<=19 years) CNS tumours: pilocytic astrocytoma (PA), anaplastic astrocytoma, glioblastoma (GBM), medulloblastoma, supratentorial CNS embryonal tumour, ependymoma, and germinoma. All patients with histologically confirmed, ICD-03 coded, first tumours, were included. Kaplan-Meier and Cox regression analyses were used to calculate hazard ratios (HR).ResultsThe total cases are as follows: males=3018 and females=2276. Highest incidence was seen in PA (n=2103). GBM displayed the worst OS, whilst PA displayed the best. Higher incidence was observed in males for all tumours, except PA. Females with ependymoma had significantly better OS compared to males, whereas males with germinomas had better OS compared to females. Females <1 year with AA had better OS than males. Increasing age significantly improved male and female survival in ependymoma and medulloblastoma.ConclusionInterrogating population-based registries such as SEER minimises bias and provides credible data. Observed differences in incidence and OS between the sexes for different paediatric CNS tumours provide useful prognostic information for clinicians. Sex genotype was a significant independent prognostic factor in ependymomas and germinomas. Further investigation of possible epigenetic and hormonal differences may provide sex-specific vulnerabilities that may be exploitable for targeted therapy.

Highlights

Distinct subtypes of central nervous system (CNS) tumours arise in the paediatric population and constitute the second most common childhood malignancy after leukaemia [1]
The exact aetiologies of paediatric CNS tumours remain largely unknown, but genetic predisposition and exposure to ionising radiation have been linked to carcinogenesis, albeit not consistently [2]
Over the 10-year period examined, pilocytic astrocytoma (PA) consistently displayed the highest incidence when compared to other CNS tumours in the paediatric population

Summary

Introduction

Distinct subtypes of central nervous system (CNS) tumours arise in the paediatric population and constitute the second most common childhood malignancy after leukaemia [1]. Previous studies in children have been conducted to investigate the influence of differences in sex on cognitive function following surgical and oncological treatment [5,6,7]. Female patients with brain tumours are at a higher risk of developing more severe neurocognitive deficits when compared to their male counterparts. It has been hypothesised that female patients with brain tumours are more susceptible to white matter damage with adjuvant treatment resulting in lower neurocognitive performance [7]. There are several population-based registries such as the US National Cancer Institute’s Surveillance, Epidemiology and End-Results (SEER) Program that report incidence and overall survival (OS) for different paediatric tumours which provide useful prognostic and demographic information for clinicians [3]

Results

Discussion

Conclusion