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    The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma.

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    Authors
    Evans, D Gareth R
    Farndon, P A
    Burnell, L D
    Gattamaneni, Rao
    Birch, Jillian M
    Affiliation
    CRC Department of Cancer Genetics, Paterson Institute for Cancer Research, Manchester, UK.
    Issue Date
    1991-11
    
    Metadata
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    Abstract
    We have investigated the incidence of Gorlin syndrome (GS) in patients with the childhood brain tumour, medulloblastoma. One hundred and seventy-three consecutive cases of medulloblastoma in the North-West Regional Health Authority between 1954 and 1989 (Manchester Regional Health Board before 1974) were studied. After review of case notes, X-rays and health surveys only 2/173 cases had evidence supporting a diagnosis of GS. A further case at 50% risk of GS died of a brain tumour aged 4 years. The incidence of GS in medulloblastoma is, therefore, probably between 1-2%. A population based study of GS in the region started in 1983 was used to assess the incidence of medulloblastoma in GS, which was found to be between 3-5%. This figure is lower than previous estimates, but this is the first population based study undertaken. In view of the early age of onset in GS (mean 2 years) children presenting with medulloblastoma, especially under 5 years, should be examined for signs of the syndrome. Those at high risk of developing multiple invasive basal cell carcinomata will then be identified.
    Citation
    The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma. 1991, 64 (5):959-61 Br. J. Cancer
    Journal
    British Journal of Cancer
    URI
    http://hdl.handle.net/10541/108463
    PubMed ID
    1931625
    Type
    Article
    Language
    en
    ISSN
    0007-0920
    Collections
    All Christie Publications
    All Paterson Institute for Cancer Research

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