Abstract:
BACKGROUND:Choroid plexus tumors (CPT) are rare, and predominate in early childhood. An association with the Li-Fraumeni syndrome (LFS) has been reported, but the biological and clinical implications of this association remain poorly defined. We have investigated the clinical features and overall survival of all CPT patients treated at Children's Hospital Los Angeles (CHLA) over a 20-year period, with particular attention to the association of CPT with LFS. METHODS:A retrospective evaluation of the course of therapy and clinical outcome was undertaken on the 42 patients diagnosed with and treated for CPT at CHLA from January 1991 to December 2010. Any association with multiple primary tumors and family histories consistent with LFS was investigated in all patients. RESULTS:Six of the 42 patients (16.7%), demonstrated either phenotypic and/or genotypic characteristics consistent with LFS, with either a distinct family history of cancer, a synchronous diagnosis of a different type of cancer, or the subsequent development of metachronous cancers. Of 11 patients with choroid plexus carcinoma tested for TP53 germline mutations, four (36.4%) were positive. A single patient with a choroid plexus papilloma had phenotypic characteristics of LFS but tested negative for TP53. CONCLUSIONS:Children with CPC appear to have a high frequency of TP53 germline mutations in association with LFS. This raises the question whether all children with CPC should be tested for TP53 germline mutations in order to institute screening to enhance early detection and treatment of subsequent cancers.
journal_name
Pediatr Blood Cancerjournal_title
Pediatric blood & cancerauthors
Gozali AE,Britt B,Shane L,Gonzalez I,Gilles F,McComb JG,Krieger MD,Lavey RS,Shlien A,Villablanca JG,Erdreich-Epstein A,Dhall G,Jubran R,Tabori U,Malkin D,Finlay JLdoi
10.1002/pbc.23349subject
Has Abstractpub_date
2012-06-01 00:00:00pages
905-9issue
6eissn
1545-5009issn
1545-5017journal_volume
58pub_type
杂志文章abstract:BACKGROUND:The introduction of the United Kingdom Medical Research Council's 10th AML trial (MRC AML 10) protocol incorporating high-dose anthracycline therapy has improved outcome of children with acute myeloid leukemia (AML). In this study, we review the results of childhood AML therapy in a Singapore university hosp...
journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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journal_title:Pediatric blood & cancer
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pub_type: 临床试验,杂志文章,多中心研究
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