An overview of the European standard clinical practice recommendations for choroid plexus tumours

David King; Brigitte Bison; Ofelia Cruz; Claudia C. Faria; Iwona Filipek; Jonathan L. Finlay; Miklós Garami; Uwe R. Kordes; Ulrike Löbel; Jilly Maclean; Torben Stamm Mikkelsen; Denise Obrecht-Sturm; Guirish Solanki; Christian Thomas; Beate Timmerman; Michal Zapotocky; Jenny Adamski

doi:10.1016/j.ejcped.2025.100226

An overview of the European standard clinical practice recommendations for choroid plexus tumours

David King, Brigitte Bison, Ofelia Cruz, Claudia C. Faria, Iwona Filipek, Jonathan L. Finlay, Miklós Garami, Uwe R. Kordes, Ulrike Löbel, Jilly Maclean, Torben Stamm Mikkelsen, Denise Obrecht-Sturm, Guirish Solanki, Christian Thomas, Beate Timmerman, Michal Zapotocky, Jenny Adamski^*

^*Corresponding author for this work

Department of Clinical Medicine - Department of Paediatrics

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaper › Review › Research › peer-review

Abstract

Choroid plexus tumours (CPT) are rare epithelial brain tumours that primarily affect very young children. They can be classified as benign choroid plexus papilloma (CPP, WHO grade 1), intermediate grade atypical choroid plexus papilloma (aCPP, WHO grade 2) or highly malignant choroid plexus carcinoma (CPC, WHO grade 3). Treatment is dependent on tumour grade, surgical resection, the age of the child and somatic and germline TP53 mutational status. CPP and aCPP have a good prognosis and are frequently curable with surgery alone. In contrast, the prognosis in CPC is often poor, despite intensive multimodal treatment with surgery, chemotherapy and radiotherapy. Management is complicated by the increased incidence of germline TP53 mutations and cancer predisposition (Li-Fraumeni syndrome), especially in CPC, whilst the rarity of the diagnosis means there are a lack of robust clinical data on which to base treatment decisions. Studies generally comprise of retrospective, single arm and centre trials or case series, and patient numbers are small leading to conflicting results that are difficult to interpret. Standardised treatment protocols have historically been lacking. The aim of this document is to provide a reference standard for the treatment of CPT on behalf of the European Society for Paediatric Oncology (SIOPE). We review the previously published literature and provide consensus key recommendations for the management of CPP, aCPP and CPC including surgical approaches and when appropriate, the role of chemotherapy and radiotherapy. Suggested follow-up and possible relapse strategies are also suggested.

Original language	English
Article number	100226
Journal	EJC Paediatric Oncology
Volume	5
ISSN	2772-610X
DOIs	https://doi.org/10.1016/j.ejcped.2025.100226
Publication status	Published - Jun 2025

Keywords

Carcinoma
Chemotherapy
Choroid plexus
Paediatric
Radiotherapy

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King, D., Bison, B., Cruz, O., Faria, C. C., Filipek, I., Finlay, J. L., Garami, M., Kordes, U. R., Löbel, U., Maclean, J., Mikkelsen, T. S., Obrecht-Sturm, D., Solanki, G., Thomas, C., Timmerman, B., Zapotocky, M., & Adamski, J. (2025). An overview of the European standard clinical practice recommendations for choroid plexus tumours. EJC Paediatric Oncology, 5, Article 100226. https://doi.org/10.1016/j.ejcped.2025.100226

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title = "An overview of the European standard clinical practice recommendations for choroid plexus tumours",

abstract = "Choroid plexus tumours (CPT) are rare epithelial brain tumours that primarily affect very young children. They can be classified as benign choroid plexus papilloma (CPP, WHO grade 1), intermediate grade atypical choroid plexus papilloma (aCPP, WHO grade 2) or highly malignant choroid plexus carcinoma (CPC, WHO grade 3). Treatment is dependent on tumour grade, surgical resection, the age of the child and somatic and germline TP53 mutational status. CPP and aCPP have a good prognosis and are frequently curable with surgery alone. In contrast, the prognosis in CPC is often poor, despite intensive multimodal treatment with surgery, chemotherapy and radiotherapy. Management is complicated by the increased incidence of germline TP53 mutations and cancer predisposition (Li-Fraumeni syndrome), especially in CPC, whilst the rarity of the diagnosis means there are a lack of robust clinical data on which to base treatment decisions. Studies generally comprise of retrospective, single arm and centre trials or case series, and patient numbers are small leading to conflicting results that are difficult to interpret. Standardised treatment protocols have historically been lacking. The aim of this document is to provide a reference standard for the treatment of CPT on behalf of the European Society for Paediatric Oncology (SIOPE). We review the previously published literature and provide consensus key recommendations for the management of CPP, aCPP and CPC including surgical approaches and when appropriate, the role of chemotherapy and radiotherapy. Suggested follow-up and possible relapse strategies are also suggested.",

keywords = "Carcinoma, Chemotherapy, Choroid plexus, Paediatric, Radiotherapy",

author = "David King and Brigitte Bison and Ofelia Cruz and Faria, {Claudia C.} and Iwona Filipek and Finlay, {Jonathan L.} and Mikl{\'o}s Garami and Kordes, {Uwe R.} and Ulrike L{\"o}bel and Jilly Maclean and Mikkelsen, {Torben Stamm} and Denise Obrecht-Sturm and Guirish Solanki and Christian Thomas and Beate Timmerman and Michal Zapotocky and Jenny Adamski",

year = "2025",

month = jun,

doi = "10.1016/j.ejcped.2025.100226",

language = "English",

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King, D, Bison, B, Cruz, O, Faria, CC, Filipek, I, Finlay, JL, Garami, M, Kordes, UR, Löbel, U, Maclean, J, Mikkelsen, TS, Obrecht-Sturm, D, Solanki, G, Thomas, C, Timmerman, B, Zapotocky, M & Adamski, J 2025, 'An overview of the European standard clinical practice recommendations for choroid plexus tumours', EJC Paediatric Oncology, vol. 5, 100226. https://doi.org/10.1016/j.ejcped.2025.100226

TY - JOUR

T1 - An overview of the European standard clinical practice recommendations for choroid plexus tumours

AU - King, David

AU - Bison, Brigitte

AU - Cruz, Ofelia

AU - Faria, Claudia C.

AU - Filipek, Iwona

AU - Finlay, Jonathan L.

AU - Garami, Miklós

AU - Kordes, Uwe R.

AU - Löbel, Ulrike

AU - Maclean, Jilly

AU - Mikkelsen, Torben Stamm

AU - Obrecht-Sturm, Denise

AU - Solanki, Guirish

AU - Thomas, Christian

AU - Timmerman, Beate

AU - Zapotocky, Michal

AU - Adamski, Jenny

PY - 2025/6

Y1 - 2025/6

N2 - Choroid plexus tumours (CPT) are rare epithelial brain tumours that primarily affect very young children. They can be classified as benign choroid plexus papilloma (CPP, WHO grade 1), intermediate grade atypical choroid plexus papilloma (aCPP, WHO grade 2) or highly malignant choroid plexus carcinoma (CPC, WHO grade 3). Treatment is dependent on tumour grade, surgical resection, the age of the child and somatic and germline TP53 mutational status. CPP and aCPP have a good prognosis and are frequently curable with surgery alone. In contrast, the prognosis in CPC is often poor, despite intensive multimodal treatment with surgery, chemotherapy and radiotherapy. Management is complicated by the increased incidence of germline TP53 mutations and cancer predisposition (Li-Fraumeni syndrome), especially in CPC, whilst the rarity of the diagnosis means there are a lack of robust clinical data on which to base treatment decisions. Studies generally comprise of retrospective, single arm and centre trials or case series, and patient numbers are small leading to conflicting results that are difficult to interpret. Standardised treatment protocols have historically been lacking. The aim of this document is to provide a reference standard for the treatment of CPT on behalf of the European Society for Paediatric Oncology (SIOPE). We review the previously published literature and provide consensus key recommendations for the management of CPP, aCPP and CPC including surgical approaches and when appropriate, the role of chemotherapy and radiotherapy. Suggested follow-up and possible relapse strategies are also suggested.

AB - Choroid plexus tumours (CPT) are rare epithelial brain tumours that primarily affect very young children. They can be classified as benign choroid plexus papilloma (CPP, WHO grade 1), intermediate grade atypical choroid plexus papilloma (aCPP, WHO grade 2) or highly malignant choroid plexus carcinoma (CPC, WHO grade 3). Treatment is dependent on tumour grade, surgical resection, the age of the child and somatic and germline TP53 mutational status. CPP and aCPP have a good prognosis and are frequently curable with surgery alone. In contrast, the prognosis in CPC is often poor, despite intensive multimodal treatment with surgery, chemotherapy and radiotherapy. Management is complicated by the increased incidence of germline TP53 mutations and cancer predisposition (Li-Fraumeni syndrome), especially in CPC, whilst the rarity of the diagnosis means there are a lack of robust clinical data on which to base treatment decisions. Studies generally comprise of retrospective, single arm and centre trials or case series, and patient numbers are small leading to conflicting results that are difficult to interpret. Standardised treatment protocols have historically been lacking. The aim of this document is to provide a reference standard for the treatment of CPT on behalf of the European Society for Paediatric Oncology (SIOPE). We review the previously published literature and provide consensus key recommendations for the management of CPP, aCPP and CPC including surgical approaches and when appropriate, the role of chemotherapy and radiotherapy. Suggested follow-up and possible relapse strategies are also suggested.

KW - Carcinoma

KW - Chemotherapy

KW - Choroid plexus

KW - Paediatric

KW - Radiotherapy

UR - http://www.scopus.com/inward/record.url?scp=105000653279&partnerID=8YFLogxK

U2 - 10.1016/j.ejcped.2025.100226

DO - 10.1016/j.ejcped.2025.100226

M3 - Review

AN - SCOPUS:105000653279

SN - 2772-610X

VL - 5

JO - EJC Paediatric Oncology

JF - EJC Paediatric Oncology

M1 - 100226

ER -

An overview of the European standard clinical practice recommendations for choroid plexus tumours

Abstract

Keywords

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