| Neurofibrosarcoma as a complication of von Recklinghausen neurofibromatosis. | |
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MedLine Citation:
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PMID: 2517529 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Neurofibrosarcoma (NFS), which is rare in the general population, occurs with excessive frequency among patients with von Recklinghausen neurofibromatosis (NF-1). Among 693 patients with NF-1 enrolled in the Baylor NF Program, 24 patients (3.5%) developed NFS or one of its variant forms. Nine of the twenty-four patients developed NFS within 3-94 months after enrolling in the Program for other reasons. Focus on these 9 patients alone suggests that the relative risk for patients with NF-1 developing NFS is 10-10,000 times greater than for the general population. Also, in NFS cohorts, patients with NF-1 are almost twice as likely to be under 30 years of age. |
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Authors:
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V M Riccardi; P P Powell |
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Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: Neurofibromatosis Volume: 2 ISSN: 1010-5662 ISO Abbreviation: Neurofibromatosis Publication Date: 1989 |
Date Detail:
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Created Date: 1990-06-14 Completed Date: 1990-06-14 Revised Date: 2005-11-16 |
Medline Journal Info:
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Nlm Unique ID: 8807525 Medline TA: Neurofibromatosis Country: SWITZERLAND |
Other Details:
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Languages: eng Pagination: 152-65 Citation Subset: IM |
Affiliation:
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Department of Medicine, Baylor College of Medicine, Houston, Tex. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Age Factors Aged Aged, 80 and over Child Child, Preschool Female Humans Infant Male Middle Aged Neurofibroma / epidemiology, etiology*, physiopathology Neurofibromatosis 1 / complications*, epidemiology, physiopathology Risk Factors |
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