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    Malignant peripheral nerve sheath tumours in neurofibromatosis 1.

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    Authors
    Evans, D Gareth R
    Baser, Michael E
    McGaughran, J
    Sharif, Saba
    Howard, E
    Moran, Anthony
    Affiliation
    University Department of Medical Genetics, St Mary's Hospital, Manchester M13 0JH, UK. Gevans@central.cmht.nwest.nhs.uk
    Issue Date
    2002-05
    
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    Abstract
    BACKGROUND: Cross sectional studies have shown that 1-2% of patients with neurofibromatosis 1 (NF1) develop malignant peripheral nerve sheath tumours (MPNST). However, no population based longitudinal studies have assessed lifetime risk. METHODS: NF1 patients with MPNST were ascertained from two sources for our north west England population of 4.1 million in the 13 year period 1984-1996: the North West Regional NF1 Register and review of notes of patients with MPNST in the North West Regional Cancer Registry. RESULTS: Twenty-one NF1 patients developed MPNST, equivalent to an annual incidence of 1.6 per 1000 and a lifetime risk of 8-13%. There were 37 patients with sporadic MPNST. The median age at diagnosis of MPNST in NF1 patients was 26 years, compared to 62 years in patients with sporadic MPNST (p<0.001). In Kaplan-Meier analyses, the five year survival from diagnosis was 21% for NF1 patients with MPNST, compared to 42% for sporadic cases of MPNST (p=0.09). One NF1 patient developed two separate MPNST in the radiation field of a previous optic glioma. CONCLUSION: The lifetime risk of MPNST in NF1 is much higher than previously estimated and warrants careful surveillance and a low threshold for investigation.
    Citation
    Malignant peripheral nerve sheath tumours in neurofibromatosis 1. 2002, 39 (5):311-4 J. Med. Genet.
    Journal
    Journal of Medical Genetics
    URI
    http://hdl.handle.net/10541/81196
    PubMed ID
    12011145
    Type
    Article
    Language
    en
    ISSN
    1468-6244
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