{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T05:19:12Z","timestamp":1789103952745,"version":"build-2803163510"},"reference-count":29,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2025,6,26]],"date-time":"2025-06-26T00:00:00Z","timestamp":1750896000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,6,26]],"date-time":"2025-06-26T00:00:00Z","timestamp":1750896000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Universit\u00e4tsklinikum Hamburg-Eppendorf (UKE)"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["J Neurooncol"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Purpose<\/jats:title>\n                    <jats:p>Choroid plexus papilloma (CPP) and atypical choroid plexus papilloma (aCPP) have excellent outcomes. However, some CPP\/aCPP relapse and may qualify for postoperative adjuvant treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>German patients from the International CPT-SIOP Registry diagnosed with CPP\/aCPP between 2011 and 2023 were included and analysed according to initial staging (postoperative residual tumor [R+], metastases [M+]), biology, postoperative treatment strategy and outcome. Additionally, patients from the published CPT-SIOP-2000 trial (PMID34997889) were combined with the registry cohort for validation purpose.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Ninety-three patients were identified (male: n\u2009=\u200953, female: n\u2009=\u200940). Median age at diagnosis was 1.9 (0.1\u201317.6) years. Initial staging was R0\/M0 in n\u2009=\u200961, R+\/M0 in n\u2009=\u200924, R0\/M\u2009+\u2009in n\u2009=\u20095 and R+\/M\u2009+\u2009in n\u2009=\u20093. aCPP was diagnosed in n\u2009=\u200938 patients. Molecular subgroup was available for n\u2009=\u200936: \u201dadult\u201d\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20093, \u201cpediatric A\u201d\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200921 and \u201cpediatric B\u201d\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200912 (6\/12 aCPP). Median follow-up was 5.5 (\u00b1\u20090.99) years. Twelve tumors relapsed: R0\/M0\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20094, R+\/M0\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20097, R+\/M\u2009+\u2009\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091. One patient with relapse died. Most patients did not receive postoperative treatment (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200988). Five patients (R0\/M\u2009+\u2009\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20092; R+\/M\u2009+\u2009\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20091; R0\/M0\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20092) received postoperative chemotherapy. None was irradiated during first-line treatment. In the enlarged cohort (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u2009197), histological diagnosis had a significant impact on PFS (5y-PFS: CPP 90\u2009\u00b1\u20093.1, aCPP 78.6\u2009\u00b1\u20094.6, PFS\u2009=\u20090.01). Both, R+ (5y-PFS: R0 90.6\u2009\u00b1\u20092.6,\n                      <jats:italic>R<\/jats:italic>\n                      \u2009+\u200969.1\u2009\u00b1\u20097.0, PFS\u2009=\u20090.01) as well as molecular subgroup \u201cpediatric B\u201d (5y-PFS: pediatric A 95.2%\u00b13.3%, pediatric B: 69.5\u2009\u00b1\u20098.6%, PFS\u2009=\u20090.02), were associated with inferior PFS, especially in aCPP.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Incomplete resection and biology impact on PFS especially in aCPP. These results extend the evidence for current stratification and treatment strategies.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s11060-025-05136-4","type":"journal-article","created":{"date-parts":[[2025,6,26]],"date-time":"2025-06-26T09:44:35Z","timestamp":1750931075000},"page":"333-343","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["CPT-SIOP registry: evaluation of risk factors for disease progression in pediatric choroid plexus papilloma"],"prefix":"10.1007","volume":"175","author":[{"given":"Christian","family":"Fiedler","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Uwe R.","family":"Kordes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Thomas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Hasselblatt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte","family":"Bison","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Behrens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"Mynarek","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolf-Dieter","family":"Kortmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beate","family":"Timmermann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Torsten","family":"Pietsch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jenny","family":"Adamski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes E A","family":"Wolff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefan","family":"Rutkowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3216-8452","authenticated-orcid":false,"given":"Denise","family":"Obrecht-Sturm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,6,26]]},"reference":[{"key":"5136_CR1","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1007\/s11060-021-03942-0","volume":"156","author":"JE Wolff","year":"2022","unstructured":"Wolff JE, Van Gool SW, Kutluk T, Diez B, Kebudi R, Timmermann B, Garami M, Sterba J, Fuller GN, Bison B, Kordes UR (2022) Final results of the choroid plexus tumor study CPT-SIOP-2000. 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