{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,16]],"date-time":"2026-08-16T09:28:06Z","timestamp":1786872486003,"version":"build-2736575974"},"reference-count":31,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2021,9,30]],"date-time":"2021-09-30T00:00:00Z","timestamp":1632960000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100005088","name":"Beijing Municipal Bureau of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100005088","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Choroid plexus carcinomas (CPCs) are rare pediatric tumors commonly associated with Li-Fraumeni syndrome (LFS), which involves a germline mutation of the tumor suppressor gene TP53.<\/jats:p><\/jats:sec><jats:sec><jats:title>Materials and Methods<\/jats:title><jats:p>We retrospectively analyzed the corresponding information of 12 cases, including the effects of surgery and radiotherapy and TP53 germline mutations, to analyse the management strategies. Kaplan-Meier curves and the log-rank test were used to evaluate the progression-free survival (PFS).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Twelve CPC patients were included, of which TP53 germline mutations were found in eight cases. All patients underwent surgical resection, and six patients received radiotherapy following with operation after initial diagnosis, one patient received radiotherapy following relapse. It was significantly different (P=0.012 and 0.028) that patients with TP53 germline mutation receiving the gross total resection (GTR) without radiotherapy showed survival advantages. Without TP53 germline mutations also showed survival advantages, but there is no statistical significance (P=0.063)<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These findings provide evidence for the therapeutic strategy that radiotherapy should not be considered for patients with TP53 germline mutations.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fonc.2021.751784","type":"journal-article","created":{"date-parts":[[2021,9,30]],"date-time":"2021-09-30T17:25:10Z","timestamp":1633022710000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":12,"title":["Choroid Plexus Carcinomas With TP53 Germline Mutations: Management and Outcome"],"prefix":"10.3389","volume":"11","author":[{"given":"Yanong","family":"Li","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hailong","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tandy","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin","family":"Feng","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yanjiao","family":"He","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chunde","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoguang","family":"Qiu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2021,9,30]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1016\/s0887-8994(02)00459-9","article-title":"Perinatal Brain Tumors: A Review of 250 Cases","volume":"27","author":"Isaacs","year":"2002","journal-title":"Pediatr Neurol"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1093\/neuonc\/noab106","article-title":"The 2021 WHO Classification of Tumors of the Central Nervous System: A Summary","volume":"23","author":"Louis","year":"2021","journal-title":"Neuro Oncol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1016\/j.jsbmb.2019.04.011","article-title":"Clinical Spectrum of Li-Fraumeni Syndrome\/Li-Fraumeni-Like Syndrome in Brazilian Individuals With the TP53 P.R337H Mutation","volume":"190","author":"Ferreira","year":"2019","journal-title":"J Steroid Biochem Mol Biol"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-019-02055-3","article-title":"An Update on the Central Nervous System Manifestations of Li-Fraumeni Syndrome","volume":"139","author":"Orr","year":"2020","journal-title":"Acta Neuropathol"},{"key":"B5","doi-asserted-by":"publisher","first-page":"1995","DOI":"10.1200\/JCO.2009.26.8169","article-title":"TP53 Alterations Determine Clinical Subgroups and Survival of Patients With Choroid Plexus Tumors","volume":"28","author":"Tabori","year":"2010","journal-title":"J Clin Oncol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1097\/CCO.0000000000000423","article-title":"Revisiting Tumor Patterns and Penetrance in Germline TP53 Mutation Carriers: Temporal Phases of Li-Fraumeni Syndrome","volume":"30","author":"Amadou","year":"2018","journal-title":"Curr Opin Oncol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.3343\/alm.2016.36.5.463","article-title":"Germline TP53 Mutation and Clinical Characteristics of Korean Patients With Li-Fraumeni Syndrome","volume":"36","author":"Park","year":"2016","journal-title":"Ann Lab Med"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuchi.2021.02.014","article-title":"Primary Intracranial Pure Endodermal Sinus Tumor: A Retrospective Series of 6 Cases in a Single Center and a Systematic Review of Overall Survival","author":"Raynald","year":"2021","journal-title":"Neurochirurgie"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.28346","article-title":"Li-Fraumeni and Li-Fraumeni-Like Syndrome Among Children Diagnosed With Pediatric Cancer in Southern Brazil","volume":"119","author":"Giacomazzi","year":"2013","journal-title":"Cancer"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2008.16.6959","article-title":"Beyond Li Fraumeni Syndrome: Clinical Characteristics of Families With P53 Germline Mutations","volume":"27","author":"Gonzalez","year":"2009","journal-title":"J Clin Oncol"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1126\/science.1140735","article-title":"An Oncogene-Induced DNA Damage Model for Cancer Development","volume":"319","author":"Halazonetis","year":"2008","journal-title":"Science"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1111\/jnc.14963","article-title":"microRNA-9-5p Alleviates Blood-Brain Barrier Damage and Neuroinflammation After Traumatic Brain Injury","volume":"153","author":"Wu","year":"2020","journal-title":"J Neurochem"},{"key":"B13","article-title":"Radiation Therapy for Choroid Plexus Carcinoma Patients With Li-Fraumeni Syndrome: Advantageous or Detrimental","volume":"35","author":"Bahar","year":"2015","journal-title":"Anticancer Res"},{"key":"B14","volume-title":"Progression-Free Survival: What Does It Mean for Psychological Well-Being or Quality of Life?","author":"Gutman","year":"2013"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1093\/jmcb\/mjz060","article-title":"P53 Modifications: Exquisite Decorations of the Powerful Guardian","volume":"11","author":"Liu","year":"2019","journal-title":"J Mol Cell Biol"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1007\/s00381-020-04885-z","article-title":"Retrospective Investigation of Hereditary Syndromes in Patients With Medulloblastoma in a Single Institution","volume":"37","author":"Wang","year":"2021","journal-title":"Childs Nerv Syst"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-020-07626-7","article-title":"Neurite Orientation Dispersion and Density Imaging Parameters may Help for the Evaluation of Epileptogenic Tubers in Tuberous Sclerosis Complex Patients","volume":"31","author":"Shao","year":"2021","journal-title":"Eur Radiol"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1038\/onc.2016.502","article-title":"Census and Evaluation of P53 Target Genes","volume":"36","author":"Fischer","year":"2017","journal-title":"Oncogene"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijrobp.2017.08.012","article-title":"Effect of Surgery, Adjuvant Therapy, and Other Prognostic Factors on Choroid Plexus Carcinoma: A Systematic Review and Individual Patient Data Analysis","volume":"99","author":"Mallick","year":"2017","journal-title":"Int J Radiat Oncol Biol Phys"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1038\/nrm2858","article-title":"Genomic Instability\u2013an Evolving Hallmark of Cancer","volume":"11","author":"Negrini","year":"2010","journal-title":"Nat Rev Mol Cell Biol"},{"key":"B21","doi-asserted-by":"publisher","first-page":"3010","DOI":"10.1016\/j.celrep.2019.08.061","article-title":"Integrated Analysis of TP53 Gene and Pathway Alterations in The Cancer Genome Atlas","volume":"28","author":"Donehower","year":"2019","journal-title":"Cell Rep"},{"key":"B22","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1007\/s40291-013-0020-0","article-title":"Tumor Protein P53 (TP53) Testing and Li-Fraumeni Syndrome: Current Status of Clinical Applications and Future Directions","volume":"17","author":"Sorrell","year":"2013","journal-title":"Mol Diagn Ther"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.23349","article-title":"Choroid Plexus Tumors; Management, Outcome, and Association With the Li-Fraumeni Syndrome: The Children's Hospital Los Angeles (CHLA) Experience, 1991-2010","volume":"58","author":"Gozali","year":"2012","journal-title":"Pediatr Blood Cancer"},{"key":"B24","doi-asserted-by":"publisher","first-page":"235","DOI":"10.1016\/j.cancergen.2019.05.002","article-title":"P53 Major Hotspot Variants Are Associated With Poorer Prognostic Features in Hereditary Cancer Patients","volume":"20","author":"Fortuno","year":"2019","journal-title":"Cancer Genet"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1038\/sj.onc.1210302","article-title":"TP53 Mutations in Human Cancers: Functional Selection and Impact on Cancer Prognosis and Outcomes","volume":"26","author":"Petitjean","year":"2007","journal-title":"Oncogene"},{"key":"B26","doi-asserted-by":"publisher","first-page":"189","DOI":"10.1097\/PDM.0b013e3182595516","article-title":"Multiplex Ligation-Dependent Probe Amplification (MLPA) in Tumor Diagnostics and Prognostics","volume":"21","author":"Homig-Holzel","year":"2012","journal-title":"Diagn Mol Pathol"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1007\/s00701-019-03832-5","article-title":"Management of Choroid Plexus Tumors-an Institutional Experience","volume":"161","author":"Hosmann","year":"2019","journal-title":"Acta Neurochir (Wien)"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1159\/000120602","article-title":"Current Management of Choroid Plexus Carcinoma in Children","volume":"17","author":"St Clair","year":"1991","journal-title":"Pediatr Neurosurg"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1007\/s11060-017-2384-1","article-title":"Choroid Plexus Tumors in Adult and Pediatric Populations: The Cleveland Clinic and University Hospitals Experience","volume":"132","author":"Bahar","year":"2017","journal-title":"J Neurooncol"},{"key":"B30","doi-asserted-by":"publisher","DOI":"10.1007\/s10143-013-0499-1","article-title":"Current Management of Choroid Plexus Carcinomas","volume":"37","author":"Sun","year":"2014","journal-title":"Neurosurg Rev"},{"key":"B31","doi-asserted-by":"publisher","DOI":"10.1097\/00006123-199803000-00006","article-title":"Choroid Plexus Carcinomas in Childhood: Clinical Features and Prognostic Factors","volume":"42","author":"Berger","year":"1998","journal-title":"Neurosurgery"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2021.751784\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,9,30]],"date-time":"2021-09-30T17:25:13Z","timestamp":1633022713000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2021.751784\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,9,30]]},"references-count":31,"alternative-id":["10.3389\/fonc.2021.751784"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2021.751784","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,9,30]]},"article-number":"751784"}}