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Final results of the Choroid Plexus Tumor study CPT-SIOP-2000

INTRODUCTION: Standards for chemotherapy against choroid plexus tumors (CPT) have not yet been established. METHODS: CPT-SIOP-2000 (NCT00500890) was an international registry for all CPT nesting a chemotherapy randomization for high-risk CPT with Carboplatin/Etoposide/Vincristine (CarbEV) versus Cyc...

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Autores principales: Wolff, Johannes E., Van Gool, Stefaan W., Kutluk, Tezer, Diez, Blanca, Kebudi, Rejin, Timmermann, Beate, Garami, Miklos, Sterba, Jaroslav, Fuller, Gregory N., Bison, Brigitte, Kordes, Uwe R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer US 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8860833/
https://www.ncbi.nlm.nih.gov/pubmed/34997889
http://dx.doi.org/10.1007/s11060-021-03942-0
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author Wolff, Johannes E.
Van Gool, Stefaan W.
Kutluk, Tezer
Diez, Blanca
Kebudi, Rejin
Timmermann, Beate
Garami, Miklos
Sterba, Jaroslav
Fuller, Gregory N.
Bison, Brigitte
Kordes, Uwe R.
author_facet Wolff, Johannes E.
Van Gool, Stefaan W.
Kutluk, Tezer
Diez, Blanca
Kebudi, Rejin
Timmermann, Beate
Garami, Miklos
Sterba, Jaroslav
Fuller, Gregory N.
Bison, Brigitte
Kordes, Uwe R.
author_sort Wolff, Johannes E.
collection PubMed
description INTRODUCTION: Standards for chemotherapy against choroid plexus tumors (CPT) have not yet been established. METHODS: CPT-SIOP-2000 (NCT00500890) was an international registry for all CPT nesting a chemotherapy randomization for high-risk CPT with Carboplatin/Etoposide/Vincristine (CarbEV) versus Cyclophosphamide/Etoposide/Vincristine (CycEV). Patients older than three years were recommended to receive irradiation: focal fields for non-metastatic CPC, incompletely resected atypical choroid plexus papilloma (APP) or metastatic choroid plexus papilloma (CPP); craniospinal fields for metastatic CPC/APP and non-responsive CPC. High risk was defined as choroid plexus carcinoma (CPC), incompletely resected APP, and all metastatic CPT. From 2000 until 2010, 158 CPT patients from 23 countries were enrolled. RESULTS: For randomized CPC, the 5/10 year progression free survival (PFS) of patients on CarbEV (n = 20) were 62%/47%, respectively, compared to 27%/18%, on CycEV (n = 15), (intention-to-treat, HR 2.6, p = 0.032). Within the registry, histological grading was the most influential prognostic factor: for CPP (n = 55) the 5/10 year overall survival (OS) and the event free survival (EFS) probabilities were 100%/97% and 92%/92%, respectively; for APP (n = 49) 96%/96% and 76%/76%, respectively; and for CPC (n = 54) 65%/51% and 41%/39%, respectively. Without irradiation, 12 out of 33 patients with CPC younger than three years were alive for a median of 8.52 years. Extent of surgery and metastases were not independent prognosticators. CONCLUSIONS: Chemotherapy for Choroid Plexus Carcinoma is feasible and effective. CarbEV is superior to CycEV. A subset of CPC can be cured without irradiation. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s11060-021-03942-0.
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spelling pubmed-88608332022-02-23 Final results of the Choroid Plexus Tumor study CPT-SIOP-2000 Wolff, Johannes E. Van Gool, Stefaan W. Kutluk, Tezer Diez, Blanca Kebudi, Rejin Timmermann, Beate Garami, Miklos Sterba, Jaroslav Fuller, Gregory N. Bison, Brigitte Kordes, Uwe R. J Neurooncol Clinical Study INTRODUCTION: Standards for chemotherapy against choroid plexus tumors (CPT) have not yet been established. METHODS: CPT-SIOP-2000 (NCT00500890) was an international registry for all CPT nesting a chemotherapy randomization for high-risk CPT with Carboplatin/Etoposide/Vincristine (CarbEV) versus Cyclophosphamide/Etoposide/Vincristine (CycEV). Patients older than three years were recommended to receive irradiation: focal fields for non-metastatic CPC, incompletely resected atypical choroid plexus papilloma (APP) or metastatic choroid plexus papilloma (CPP); craniospinal fields for metastatic CPC/APP and non-responsive CPC. High risk was defined as choroid plexus carcinoma (CPC), incompletely resected APP, and all metastatic CPT. From 2000 until 2010, 158 CPT patients from 23 countries were enrolled. RESULTS: For randomized CPC, the 5/10 year progression free survival (PFS) of patients on CarbEV (n = 20) were 62%/47%, respectively, compared to 27%/18%, on CycEV (n = 15), (intention-to-treat, HR 2.6, p = 0.032). Within the registry, histological grading was the most influential prognostic factor: for CPP (n = 55) the 5/10 year overall survival (OS) and the event free survival (EFS) probabilities were 100%/97% and 92%/92%, respectively; for APP (n = 49) 96%/96% and 76%/76%, respectively; and for CPC (n = 54) 65%/51% and 41%/39%, respectively. Without irradiation, 12 out of 33 patients with CPC younger than three years were alive for a median of 8.52 years. Extent of surgery and metastases were not independent prognosticators. CONCLUSIONS: Chemotherapy for Choroid Plexus Carcinoma is feasible and effective. CarbEV is superior to CycEV. A subset of CPC can be cured without irradiation. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s11060-021-03942-0. Springer US 2022-01-08 2022 /pmc/articles/PMC8860833/ /pubmed/34997889 http://dx.doi.org/10.1007/s11060-021-03942-0 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Clinical Study
Wolff, Johannes E.
Van Gool, Stefaan W.
Kutluk, Tezer
Diez, Blanca
Kebudi, Rejin
Timmermann, Beate
Garami, Miklos
Sterba, Jaroslav
Fuller, Gregory N.
Bison, Brigitte
Kordes, Uwe R.
Final results of the Choroid Plexus Tumor study CPT-SIOP-2000
title Final results of the Choroid Plexus Tumor study CPT-SIOP-2000
title_full Final results of the Choroid Plexus Tumor study CPT-SIOP-2000
title_fullStr Final results of the Choroid Plexus Tumor study CPT-SIOP-2000
title_full_unstemmed Final results of the Choroid Plexus Tumor study CPT-SIOP-2000
title_short Final results of the Choroid Plexus Tumor study CPT-SIOP-2000
title_sort final results of the choroid plexus tumor study cpt-siop-2000
topic Clinical Study
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8860833/
https://www.ncbi.nlm.nih.gov/pubmed/34997889
http://dx.doi.org/10.1007/s11060-021-03942-0
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