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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.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Results<\/jats:title>\n            <jats:p>For randomized CPC, the 5\/10\u00a0year progression free survival (PFS) of patients on CarbEV (n\u2009=\u200920) were 62%\/47%, respectively, compared to 27%\/18%, on CycEV (n\u2009=\u200915), (intention-to-treat, HR 2.6, p\u2009=\u20090.032). Within the registry, histological grading was the most influential prognostic factor: for CPP (n\u2009=\u200955) the 5\/10\u00a0year overall survival (OS) and the event free survival (EFS) probabilities were 100%\/97% and 92%\/92%, respectively; for APP (n\u2009=\u200949) 96%\/96% and 76%\/76%, respectively; and for CPC (n\u2009=\u200954) 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\u00a0years. Extent of surgery and metastases were not independent prognosticators.<\/jats:p>\n          <\/jats:sec>\n          <jats:sec>\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p>Chemotherapy for Choroid Plexus Carcinoma is feasible and effective. CarbEV is superior to CycEV. A subset of CPC can be cured without irradiation.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1007\/s11060-021-03942-0","type":"journal-article","created":{"date-parts":[[2022,1,8]],"date-time":"2022-01-08T02:02:35Z","timestamp":1641607355000},"page":"599-613","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":33,"title":["Final results of the Choroid Plexus Tumor study CPT-SIOP-2000"],"prefix":"10.1007","volume":"156","author":[{"given":"Johannes E.","family":"Wolff","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8102-5968","authenticated-orcid":false,"given":"Stefaan W.","family":"Van Gool","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1918-4407","authenticated-orcid":false,"given":"Tezer","family":"Kutluk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7115-7000","authenticated-orcid":false,"given":"Blanca","family":"Diez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4344-8174","authenticated-orcid":false,"given":"Rejin","family":"Kebudi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2274-2008","authenticated-orcid":false,"given":"Beate","family":"Timmermann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4298-2746","authenticated-orcid":false,"given":"Miklos","family":"Garami","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8482-8352","authenticated-orcid":false,"given":"Jaroslav","family":"Sterba","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9447-2647","authenticated-orcid":false,"given":"Gregory N.","family":"Fuller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5849-8671","authenticated-orcid":false,"given":"Brigitte","family":"Bison","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6375-2320","authenticated-orcid":false,"given":"Uwe R.","family":"Kordes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,1,8]]},"reference":[{"key":"3942_CR1","unstructured":"Kaatsch P GD, Spix C (2019) German Childhood Cancer Registry\u2014Annual Report 2018 (1980\u20132017). https:\/\/www.kinderkrebsregister.de\/dkkr-gb\/latest-publications\/annual-reports.html?L=1."},{"key":"3942_CR2","doi-asserted-by":"publisher","first-page":"1231","DOI":"10.1093\/neuonc\/noab106","volume":"23","author":"DN Louis","year":"2021","unstructured":"Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, Hawkins C, Ng HK, Pfister SM, Reifenberger G, Soffietti R, von Deimling A, Ellison DW (2021) The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. 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