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@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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