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.
References
Berger C, Thiesse P, Lellouch-Tubiana A et al (1998) Choroid plexus carcinomas in childhood: clinical features and prognostic factors. Neurosurgery 42:470–475
WHO classification of tumors of the Central Nervous System (2016) WHO/IARC Classification of Tumors, Revised 4th Edition, 1; 124–129
Jeibmann A, Hasselbatt M, Gerss J et al (2006) Prognostic implications of atypical histological features in choroid plexus papilloma. J Neuropathol Exp Neurol 65(11):1069–1073
Wrede B, Hasselblatt M et al (2009) Atypical choroid plexus papilloma: clinical experience in the CPT-SIOP-2000 study. J Neurooncol 95(3):383–392
Scala M, Morana G et al (2017) Atypical choroid plexus papillomas: spontaneous resolution of diffuse leptomeningeal contrast enhancement after primary tumor removal in 2 pediatric cases. J Neurosurg Pediatr 20(3):284–288
Louis DN, Ohgaki H, Wiestler OD, Cavenee WK (2016) WHO classification of the central nervous system; Revised 4th Edition. IARC
Mansouri S, Suppiah S, Mamatjan Y et al (2021) Epigenomic, genomic, and transcriptomic landscape of schwannomatosis. Acta Neuropathol 141:101–116. https://doi.org/10.1007/s00401-020-02230-x
Cavalli F, Hübner J, Sharma T et al (2018) Heterogeneity within the PF-EPN-B ependymoma subgroup. Acta Neuropathol 136:227–237. https://doi.org/10.1007/s00401-018-1888-x
Amer N, Taha H, Hesham D et al (2021) Aggresomes predict poor outcomes and implicate proteostasis in the pathogenesis of pediatric choroid plexus tumors. J Neurooncol 52:67–78
Capper D, Jones D, Sill M et al (2018) DNA methylation-based classification of central nervous system tumours. Nature 555:469–474. https://doi.org/10.1038/nature26000 (Epub 2018 Mar 14)
Bahar M, Hashem H, Tekautz T et al (2017) Choroid plexus tumors in adult and pediatric populations: the Cleveland clinic and university hospitals experience. J Neurooncol 132(3):427–432
Lafay-Cousin L, Keene D, Carret A et al (2011) Choroid plexus tumours in children less than 36 months: the Canadian pediatric brain tumour consortium (CPBTC) experience. Childs Nerv Syst 27:259–264
Wolff J, Sajedi M, Brandt R et al (2002) Choroid plexus tumours. Br J Cancer 87(10):1086–1091
Thomas C, Ruland V, Kordes U et al (2015) Pediatric atypical choroid plexus papilloma reconsidered: increased mitotic activity is prognostic in only older children. Acta Neuropathol 129(6):925–927
Thomas C, Sill M, Ruland V et al (2016) Methylation profiling of choroid plexus tumors reveals 3 clinically distinct subgroups. Neuro Oncol 18(6):790–796
Merino D, Shlien A, Vilani A et al (2015) Molecular characterization of choroid plexus tumors reveals novel clinically relevant subgroups. Clin Cancer Res 21(1):184–192
Japp A, Gessi M, Messing-Junger M et al (2015) High-resolution genomic analysis does not qualify atypical plexus papilloma as a separate entity among choroid plexus tumors. J Neuropathol Exp Neurol 74(2):110–120
Pienkowska M, Choufani S, Turinsky A et al (2019) DNA methylation signature is prognostic of choroid plexus tumour aggressiveness. Clin Epigenetics 11(1):117. https://doi.org/10.1186/s13148-019-0708-z
Tabori U, Shlien A, Baskin B et al (2010) TP53 Alterations determine clinical subgroups and survival of patients with choroid plexus tumours. J Clin Oncol 28(12):1995–2001
Siegfried A, Munzer MS et al (2017) A French retrospective study on clinical outcome in 102 choroid plexus tumors in children. J Neurooncol 135:151–160
Hosmann A, Hinker F, Dorfer C et al (2019) Management of choroid plexus tumors-an institutional experience. Acta Neurochir (Wien) 161(4):745–754. https://doi.org/10.1007/s00701-019-03832-5
Zhou WJ, Wang X, Peng JY et al (2018) Clinical features and prognostic risk factors of choroid plexus tumors in children. Chin Med J (Engl) 131(24):2938–2946. https://doi.org/10.4103/0366-6999.247195
Dash C, Moorthy S, Garg K et al (2019) Management of choroid plexus tumors in infants and young children up to 4 years of age: an institutional experience. World Neurosurg 121:e237–e245. https://doi.org/10.1016/j.wneu.2018.09.089
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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.
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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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DOI: https://doi.org/10.1007/s11060-021-03843-2