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

J 2022

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

WOLFF, Johannes E., W. VAN GOOL STEFAAN, Tezer KUTLUK, Blanca DIEZ, Rejin KEBUDI et. al.

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

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30204 Oncology

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

References:

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

Abstract

V originále

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 MU

Name: Personalizovaná léčba v dětské onkologii: na cestě k „liquid dynamic medicine“ a „N-of-1 clinical trials“

Investor: Masaryk University

Citovat

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.

@article{2251436,
   author = {Wolff, Johannes E. and Van Gool Stefaan, W. and Kutluk, Tezer and Diez, Blanca and Kebudi, Rejin and Timmermann, Beate and Garami, Miklos and Štěrba, Jaroslav and Fuller, Gregory N. and Bison, Brigitte and Kordes, Uwe R.},
   article_location = {New York},
   article_number = {3},
   doi = {http://dx.doi.org/10.1007/s11060-021-03942-0},
   keywords = {Choroid plexus tumors; Chemotherapy; Irradiation; Li-Fraumeni syndrome},
   language = {eng},
   issn = {0167-594X},
   journal = {Journal of Neuro-Oncology},
   title = {Final results of the Choroid Plexus Tumor study CPT-SIOP-2000},
   url = {https://link.springer.com/article/10.1007/s11060-021-03942-0},
   volume = {156},
   year = {2022}
}

TY  - JOUR
ID  - 2251436
AU  - Wolff, Johannes E. - Van Gool Stefaan, W. - Kutluk, Tezer - Diez, Blanca - Kebudi, Rejin - Timmermann, Beate - Garami, Miklos - Štěrba, Jaroslav - Fuller, Gregory N. - Bison, Brigitte - Kordes, Uwe R.
PY  - 2022
TI  - Final results of the Choroid Plexus Tumor study CPT-SIOP-2000
JF  - Journal of Neuro-Oncology
VL  - 156
IS  - 3
SP  - 599-613
EP  - 599-613
PB  - Springer
SN  - 0167594X
KW  - Choroid plexus tumors
KW  - Chemotherapy
KW  - Irradiation
KW  - Li-Fraumeni syndrome
UR  - https://link.springer.com/article/10.1007/s11060-021-03942-0
N2  - 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.
ER  -

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. \textit{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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