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Polish Multi-Institutional Study of Children with Ependymoma—Clinical Practice Outcomes in the Light of Prospective Trials

We performed a multi-institutional analysis of 74 children with ependymoma to evaluate to what extent the clinical outcome of prospective trials could be reproduced in routine practice. The evaluation of factors that correlated with outcome was performed with a log rank test and a Cox proportional-h...

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Autores principales: Napieralska, Aleksandra, Mizia-Malarz, Agnieszka, Stolpa, Weronika, Pawłowska, Ewa, Krawczyk, Małgorzata A., Konat-Bąska, Katarzyna, Kaczorowska, Aneta, Brąszewski, Arkadiusz, Harat, Maciej
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8700631/
https://www.ncbi.nlm.nih.gov/pubmed/34943596
http://dx.doi.org/10.3390/diagnostics11122360
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author Napieralska, Aleksandra
Mizia-Malarz, Agnieszka
Stolpa, Weronika
Pawłowska, Ewa
Krawczyk, Małgorzata A.
Konat-Bąska, Katarzyna
Kaczorowska, Aneta
Brąszewski, Arkadiusz
Harat, Maciej
author_facet Napieralska, Aleksandra
Mizia-Malarz, Agnieszka
Stolpa, Weronika
Pawłowska, Ewa
Krawczyk, Małgorzata A.
Konat-Bąska, Katarzyna
Kaczorowska, Aneta
Brąszewski, Arkadiusz
Harat, Maciej
author_sort Napieralska, Aleksandra
collection PubMed
description We performed a multi-institutional analysis of 74 children with ependymoma to evaluate to what extent the clinical outcome of prospective trials could be reproduced in routine practice. The evaluation of factors that correlated with outcome was performed with a log rank test and a Cox proportional-hazard model. Survival was estimated with the Kaplan–Meier method. The majority of patients had brain tumours (89%). All had surgery as primary treatment, with adjuvant radiotherapy (RTH) and chemotherapy (CTH) applied in 78% and 57%, respectively. Median follow-up was 80 months and 18 patients died. Five- and 10-year overall survival (OS) was 83% and 73%. Progression was observed in 32 patients, with local recurrence in 28 cases. The presence of metastases was a negative prognostic factor for OS. Five- and 10-year progression-free survival (PFS) was 55% and 40%, respectively. The best outcome in patients with non-disseminated brain tumours was observed when surgery was followed by RTH (+/−CTH afterwards; p = 0.0001). Children under 3 years old who received RTH in primary therapy had better PFS (p = 0.010). The best outcome of children with ependymoma is observed in patients who received radical surgery followed by RTH, and irradiation should not be omitted in younger patients. The role of CTH remains debatable.
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spelling pubmed-87006312021-12-24 Polish Multi-Institutional Study of Children with Ependymoma—Clinical Practice Outcomes in the Light of Prospective Trials Napieralska, Aleksandra Mizia-Malarz, Agnieszka Stolpa, Weronika Pawłowska, Ewa Krawczyk, Małgorzata A. Konat-Bąska, Katarzyna Kaczorowska, Aneta Brąszewski, Arkadiusz Harat, Maciej Diagnostics (Basel) Article We performed a multi-institutional analysis of 74 children with ependymoma to evaluate to what extent the clinical outcome of prospective trials could be reproduced in routine practice. The evaluation of factors that correlated with outcome was performed with a log rank test and a Cox proportional-hazard model. Survival was estimated with the Kaplan–Meier method. The majority of patients had brain tumours (89%). All had surgery as primary treatment, with adjuvant radiotherapy (RTH) and chemotherapy (CTH) applied in 78% and 57%, respectively. Median follow-up was 80 months and 18 patients died. Five- and 10-year overall survival (OS) was 83% and 73%. Progression was observed in 32 patients, with local recurrence in 28 cases. The presence of metastases was a negative prognostic factor for OS. Five- and 10-year progression-free survival (PFS) was 55% and 40%, respectively. The best outcome in patients with non-disseminated brain tumours was observed when surgery was followed by RTH (+/−CTH afterwards; p = 0.0001). Children under 3 years old who received RTH in primary therapy had better PFS (p = 0.010). The best outcome of children with ependymoma is observed in patients who received radical surgery followed by RTH, and irradiation should not be omitted in younger patients. The role of CTH remains debatable. MDPI 2021-12-14 /pmc/articles/PMC8700631/ /pubmed/34943596 http://dx.doi.org/10.3390/diagnostics11122360 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Napieralska, Aleksandra
Mizia-Malarz, Agnieszka
Stolpa, Weronika
Pawłowska, Ewa
Krawczyk, Małgorzata A.
Konat-Bąska, Katarzyna
Kaczorowska, Aneta
Brąszewski, Arkadiusz
Harat, Maciej
Polish Multi-Institutional Study of Children with Ependymoma—Clinical Practice Outcomes in the Light of Prospective Trials
title Polish Multi-Institutional Study of Children with Ependymoma—Clinical Practice Outcomes in the Light of Prospective Trials
title_full Polish Multi-Institutional Study of Children with Ependymoma—Clinical Practice Outcomes in the Light of Prospective Trials
title_fullStr Polish Multi-Institutional Study of Children with Ependymoma—Clinical Practice Outcomes in the Light of Prospective Trials
title_full_unstemmed Polish Multi-Institutional Study of Children with Ependymoma—Clinical Practice Outcomes in the Light of Prospective Trials
title_short Polish Multi-Institutional Study of Children with Ependymoma—Clinical Practice Outcomes in the Light of Prospective Trials
title_sort polish multi-institutional study of children with ependymoma—clinical practice outcomes in the light of prospective trials
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8700631/
https://www.ncbi.nlm.nih.gov/pubmed/34943596
http://dx.doi.org/10.3390/diagnostics11122360
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