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

Varování

Publikace nespadá pod Ekonomicko-správní fakultu, ale pod Lékařskou fakultu. Oficiální stránka publikace je na webu muni.cz.

Autoři	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.
Rok publikování	2022
Druh	Článek v odborném periodiku
Časopis / Zdroj	Journal of Neuro-Oncology
Fakulta / Pracoviště MU	Lékařská fakulta
Citace
www	https://link.springer.com/article/10.1007/s11060-021-03942-0
Doi	http://dx.doi.org/10.1007/s11060-021-03942-0
Klíčová slova	Choroid plexus tumors; Chemotherapy; Irradiation; Li-Fraumeni syndrome
Popis	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.
Související projekty:	Personalizovaná léčba v dětské onkologii: na cestě k „liquid dynamic medicine“ a „N-of-1 clinical trials“