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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
5
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pubmed:dateCreated |
2002-7-26
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pubmed:abstractText |
Childhood atypical teratoid/rhabdoid tumor (AT/RT) of the central nervous system (CNS) is a recently described entity. Diagnosis is based on distinctive light microscopy and immunohistochemical findings, coupled with molecular genetic analysis. Most AT/RTs demonstrate monosomy 22 or deletions of chromosome band 22q11 with alterations of the hSNF5/INI1 gene. The tumor's incidence is still undefined, but it may comprise as high as 1 in 4 primitive CNS tumors in infants. Treatment is far from optimal, but there are occasional long-term survivors, especially among older children. Therapeutic approached have included surgery, chemotherapy, and radiotherapy. Prospective clinical trials are needed for children with AT/RTs.
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
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pubmed:status |
MEDLINE
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pubmed:issn |
1077-4114
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pubmed:author |
pubmed-author:BiegelJaclyn AJA,
pubmed-author:BlaneySusanS,
pubmed-author:FinlayJonathanJ,
pubmed-author:GeyerJ RussellJR,
pubmed-author:HeidemanRichardR,
pubmed-author:HildenJoanneJ,
pubmed-author:JanssAnna JAJ,
pubmed-author:KunLarryL,
pubmed-author:PackerRoger JRJ,
pubmed-author:RorkeLucy BLB,
pubmed-author:SmithMalcolmM,
pubmed-author:VezinaGilbertG
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pubmed:issnType |
Print
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pubmed:volume |
24
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
337-42
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pubmed:dateRevised |
2011-10-6
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pubmed:meshHeading |
pubmed-meshheading:12142780-Antineoplastic Agents,
pubmed-meshheading:12142780-Brain Neoplasms,
pubmed-meshheading:12142780-Child, Preschool,
pubmed-meshheading:12142780-Chromosomal Proteins, Non-Histone,
pubmed-meshheading:12142780-Chromosome Deletion,
pubmed-meshheading:12142780-Chromosomes, Human, Pair 22,
pubmed-meshheading:12142780-DNA-Binding Proteins,
pubmed-meshheading:12142780-General Surgery,
pubmed-meshheading:12142780-Humans,
pubmed-meshheading:12142780-Infant,
pubmed-meshheading:12142780-Monosomy,
pubmed-meshheading:12142780-Neoplasm Proteins,
pubmed-meshheading:12142780-Radiotherapy,
pubmed-meshheading:12142780-Rhabdoid Tumor,
pubmed-meshheading:12142780-Teratoma,
pubmed-meshheading:12142780-Transcription Factors
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pubmed:articleTitle |
Atypical teratoid/rhabdoid tumor of the central nervous system: report on workshop.
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pubmed:affiliation |
Department of Neurology, Children's National Medical Center, The George Washington University Medical Center, Washington, DC 20010, USA. rpacker@cnmc.org
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pubmed:publicationType |
Research Support, U.S. Gov't, P.H.S.,
Congresses,
Research Support, Non-U.S. Gov't
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