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.

Základní údaje

Originální název

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

Autoři

WOLFF, Johannes E., W. VAN GOOL STEFAAN, Tezer KUTLUK, Blanca DIEZ, Rejin KEBUDI, Beate TIMMERMANN, Miklos GARAMI, Jaroslav ŠTĚRBA (203 Česká republika, domácí), Gregory N. FULLER, Brigitte BISON a Uwe R. KORDES (garant)

Vydání

Journal of Neuro-Oncology, New York, Springer, 2022, 0167-594X

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30204 Oncology

Stát vydavatele

Spojené státy

Utajení

není předmětem státního či obchodního tajemství

Odkazy

Impakt faktor

Impact factor: 3.900

Kód RIV

RIV/00216224:14110/22:00128441

Organizační jednotka

Lékařská fakulta

UT WoS

000740391400001

Klíčová slova anglicky

Choroid plexus tumors; Chemotherapy; Irradiation; Li-Fraumeni syndrome

Štítky

Příznaky

Mezinárodní význam, Recenzováno
Změněno: 16. 2. 2023 14:34, Mgr. Tereza Miškechová

Anotace

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.

Návaznosti

MUNI/A/1427/2021, interní kód MU
Název: Personalizovaná léčba v dětské onkologii: na cestě k „liquid dynamic medicine“ a „N-of-1 clinical trials“
Investor: Masarykova univerzita, Personalizovaná léčba v dětské onkologii: na cestě k „liquid dynamic medicine“ a „N-of-1 clinical trials“