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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
4
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pubmed:dateCreated |
2008-7-23
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pubmed:abstractText |
People with neurofibromatosis 1 (NF1) have multiple benign neurofibromas and a 10% lifetime risk of developing malignant peripheral nerve sheath tumors (MPNSTs). Most MPNSTs develop from benign plexiform neurofibromas, so the burden of benign tumors may be a risk factor for developing MPNST. We studied 13 NF1 patients with MPNSTs and 26 age- and sex-matched controls (NF1 patients who did not have MPNSTs) with detailed clinical examinations and whole-body MRI to characterize their body burden of internal benign neurofibromas. Internal plexiform neurofibromas were identified in 22 (56%) of the 39 NF1 patients studied. All six of the NF1 patients with MPNSTs under 30 years of age had neurofibromas visualized on whole-body MRI, compared to only 3 of 11 matched NF1 controls under age 30 (p < 0.05). Both the median number of plexiform neurofibromas (p < 0.05) and the median neurofibroma volume (p < 0.01) on whole-body MRI were significantly greater among MPNST patients younger than 30 years of age than among controls. No significant differences in whole-body MRI findings were observed between NF1 patients with MPNSTs and controls who were 30 years of age or older. Whole-body MRI of NF1 patients allows assessment of the burden of internal neurofibromas, most of which are not apparent on physical examination. Whole-body imaging of young NF1 patients may allow those at highest risk for developing MPNST to be identified early in life. Close surveillance of these high-risk patients may permit earlier diagnosis and more effective treatment of MPNSTs that develop.
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pubmed:commentsCorrections |
http://linkedlifedata.com/resource/pubmed/commentcorrection/18559970-10525761,
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http://linkedlifedata.com/resource/pubmed/commentcorrection/18559970-9633723
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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:status |
MEDLINE
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pubmed:month |
Aug
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pubmed:issn |
1522-8517
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
10
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
593-8
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pubmed:dateRevised |
2009-11-18
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pubmed:meshHeading |
pubmed-meshheading:18559970-Adolescent,
pubmed-meshheading:18559970-Adult,
pubmed-meshheading:18559970-Child,
pubmed-meshheading:18559970-Child, Preschool,
pubmed-meshheading:18559970-Female,
pubmed-meshheading:18559970-Humans,
pubmed-meshheading:18559970-Infant,
pubmed-meshheading:18559970-Magnetic Resonance Imaging,
pubmed-meshheading:18559970-Male,
pubmed-meshheading:18559970-Middle Aged,
pubmed-meshheading:18559970-Neurofibromatosis 1,
pubmed-meshheading:18559970-Tumor Burden,
pubmed-meshheading:18559970-Whole Body Imaging
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pubmed:year |
2008
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pubmed:articleTitle |
Assessment of benign tumor burden by whole-body MRI in patients with neurofibromatosis 1.
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pubmed:affiliation |
Department of Maxillofacial Surgery, University Hospital Eppendorf, Hamburg, Germany.
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pubmed:publicationType |
Journal Article
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