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    Second primary tumors in neurofibromatosis 1 patients treated for optic glioma: substantial risks after radiotherapy.

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    Authors
    Sharif, Saba
    Ferner, Rosalie
    Birch, Jillian M
    Gillespie, James E
    Gattamaneni, Rao
    Baser, Michael E
    Evans, D Gareth R
    Affiliation
    Department of Clinical Genetics, St Mary's Hospital, Manchester, United Kingdom.
    Issue Date
    2006-06-01
    
    Metadata
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    Abstract
    PURPOSE: Optic pathway gliomas (OPGs) are the most common CNS tumor in neurofibromatosis 1 (NF1) patients. We evaluated the long-term risk of second tumors in NF1-related OPGs after radiotherapy. PATIENTS AND METHODS: We reviewed 80 NF1 OPG patients from two NF1 clinics to evaluate the long-term risk of developing subsequent nervous system tumors, with or without radiotherapy. RESULTS: Fifty-eight patients were assessable for second tumors. Nine (50%) of 18 patients who received radiotherapy after their OPGs developed 12 second tumors in 308 person-years of follow-up after radiotherapy. Eight (20%) of 40 patients who were not treated with radiotherapy developed nine tumors in 721 person-years of follow-up after diagnosis of their OPGs. The relative risk of second nervous system tumor after radiotherapy was 3.04 (95% CI, 1.29 to 7.15). CONCLUSION: There is a significantly increased risk of second nervous system tumors in those NF1 patients who received radiotherapy for their OPGs, especially when treated in childhood. Thus radiotherapy should only be used if absolutely essential in children with NF1.
    Citation
    Second primary tumors in neurofibromatosis 1 patients treated for optic glioma: substantial risks after radiotherapy. 2006, 24 (16):2570-5 J. Clin. Oncol.
    Journal
    Journal of Clinical Oncology
    URI
    http://hdl.handle.net/10541/72619
    DOI
    10.1200/JCO.2005.03.8349
    PubMed ID
    16735710
    Type
    Article
    Language
    en
    ISSN
    1527-7755
    ae974a485f413a2113503eed53cd6c53
    10.1200/JCO.2005.03.8349
    Scopus Count
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