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Paediatric atypical choroid plexus papilloma: is adjuvant therapy necessary?

Abstract

Introduction

Choroid Plexus Tumours (CPTs) account for 1–4% of all brain tumours in children. Atypical choroid plexus papillomas (aCPPs) are a subset of these tumours, defined over a decade ago, yet no consensus exists on the optimal approach to their management.

Methods

We conducted a retrospective analysis of all patients treated for CPTs at the Hospital for Sick Children between January 1, 2000, and December 31, 2018, and focused on patients with aCPP. Data extracted from the patient records for analysis included: demographic and clinical features, radiological imaging, surgical and adjuvant therapies, key pathological features, immunohistochemical staining for TP53 and tumour karyotype. Six of seven aCPP samples were profiled using Illumina HumanMethylationEPIC arrays and the top 10,000 most variably methylated probes were visualized using tSNE. Copy number inferencing was also performed.

Results

Twenty-nine patients were diagnosed with CPT, seven of whom had a diagnosis of aCPP as confirmed by histological review. Methylation profiling demonstrated that aCPPs clustered with both choroid plexus papillomas (CPPs) and choroid plexus carcinomas (CPCs). Complete resection of the tumour was pursued in all cases of aCPP and no patient received adjuvant therapy. All aCPP patients were alive at last follow up.

Conclusions

This limited case series suggests that paediatric aCPP can be successfully managed with surgical resection alone, followed by a ‘watch and wait’ approach thus avoiding adjuvant therapies. A deeper understanding of the biology of aCPP is required to identify objective markers which can help provide robust risk stratification and inform treatment strategies.

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Data Availability

The data that support the findings of this study are available from the corresponding author upon reasonable request.

Code availability

Not applicable.

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The authors received no financial support for this research, authorship and/or publications of this article.

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All authors contributed to the study conception and design. Material preparation, data collection and analysis were performed by CB-F, L-NH, YM, GZ, VR and UB. The first draft of the manuscript was written by CB-F and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Chantelle Browne-Farmer.

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Browne-Farmer, C., Hazrati, LN., Mamatjan, Y. et al. Paediatric atypical choroid plexus papilloma: is adjuvant therapy necessary?. J Neurooncol 155, 63–70 (2021). https://doi.org/10.1007/s11060-021-03843-2

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Keywords

  • Paediatric atypical choroid plexus papilloma
  • Choroid plexus tumour
  • Management
  • Outcomes