Malignant peripheral nerve sheath tumours in neurofibromatosis 1.

DDGR Evans, M Baser, J McGaughran, S Sharif, E Howard, A. Moran

    Research output: Contribution to journalArticlepeer-review

    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
    Original languageEnglish
    Pages (from-to)311-4
    JournalJ Med Genet
    Volume39( 5)
    Publication statusPublished - May 2002

    Keywords

    • Adolescent
    • Adult
    • Aged
    • epidemiology: England
    • Female
    • Humans
    • Incidence
    • Longitudinal Studies
    • Male
    • Middle Aged
    • diagnosis: Neurofibromatosis 1
    • Risk Assessment
    • Survival Rate

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