WOLFF, Johannes E., W. VAN GOOL STEFAAN, Tezer KUTLUK, Blanca DIEZ, Rejin KEBUDI, Beate TIMMERMANN, Miklos GARAMI, Jaroslav ŠTĚRBA, Gregory N. FULLER, Brigitte BISON and Uwe R. KORDES. Final results of the Choroid Plexus Tumor study CPT-SIOP-2000. Journal of Neuro-Oncology. New York: Springer, 2022, vol. 156, No 3, p. 599-613. ISSN 0167-594X. Available from: https://dx.doi.org/10.1007/s11060-021-03942-0.
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Basic information
Original name Final results of the Choroid Plexus Tumor study CPT-SIOP-2000
Authors WOLFF, Johannes E., W. VAN GOOL STEFAAN, Tezer KUTLUK, Blanca DIEZ, Rejin KEBUDI, Beate TIMMERMANN, Miklos GARAMI, Jaroslav ŠTĚRBA (203 Czech Republic, belonging to the institution), Gregory N. FULLER, Brigitte BISON and Uwe R. KORDES (guarantor).
Edition Journal of Neuro-Oncology, New York, Springer, 2022, 0167-594X.
Other information
Original language English
Type of outcome Article in a journal
Field of Study 30204 Oncology
Country of publisher United States of America
Confidentiality degree is not subject to a state or trade secret
WWW URL
Impact factor Impact factor: 3.900
RIV identification code RIV/00216224:14110/22:00128441
Organization unit Faculty of Medicine
Doi http://dx.doi.org/10.1007/s11060-021-03942-0
UT WoS 000740391400001
Keywords in English Choroid plexus tumors; Chemotherapy; Irradiation; Li-Fraumeni syndrome
Tags 14110321, rivok
Tags International impact, Reviewed
Changed by Changed by: Mgr. Tereza Miškechová, učo 341652. Changed: 16/2/2023 14:34.
Abstract
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.
Links
MUNI/A/1427/2021, interní kód MUName: Personalizovaná léčba v dětské onkologii: na cestě k „liquid dynamic medicine“ a „N-of-1 clinical trials“
Investor: Masaryk University
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