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

Kód výsledku v IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00064203%3A_____%2F25%3A10496727" target="_blank" >RIV/00064203:_____/25:10496727 - isvavai.cz</a>

Nalezeny alternativní kódy

RIV/00216208:11130/25:10496727

Výsledek na webu

<a href="https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=4Wqr5lQBzI" target="_blank" >https://verso.is.cuni.cz/pub/verso.fpl?fname=obd_publikace_handle&handle=4Wqr5lQBzI</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1016/j.ejcped.2025.100226" target="_blank" >10.1016/j.ejcped.2025.100226</a>

Jazyk výsledku

angličtina

Název v původním jazyce

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

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

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

Popis výsledku anglicky

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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30204 - Oncology

Projekt

—

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2025

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

EJC Paediatric Oncology

ISSN

2772-610X

e-ISSN

2772-610X

Svazek periodika

5

Číslo periodika v rámci svazku

June

Stát vydavatele periodika

GB - Spojené království Velké Británie a Severního Irska

Počet stran výsledku

13

Strana od-do

100226

Kód UT WoS článku

001592225000005

EID výsledku v databázi Scopus

2-s2.0-105000653279