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dc.contributor.authorEvans, D Gareth Ren
dc.contributor.authorFarndon, P Aen
dc.contributor.authorBurnell, L Den
dc.contributor.authorGattamaneni, Raoen
dc.contributor.authorBirch, Jillian Men
dc.date.accessioned2010-07-27T15:51:21Z
dc.date.available2010-07-27T15:51:21Z
dc.date.issued1991-11
dc.identifier.citationThe incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma. 1991, 64 (5):959-61 Br. J. Canceren
dc.identifier.issn0007-0920
dc.identifier.pmid1931625
dc.identifier.urihttp://hdl.handle.net/10541/108463
dc.description.abstractWe 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.
dc.language.isoenen
dc.subjectCerebellar Canceren
dc.subject.meshBasal Cell Nevus Syndrome
dc.subject.meshCerebellar Neoplasms
dc.subject.meshChild
dc.subject.meshChild, Preschool
dc.subject.meshFamily Health
dc.subject.meshFemale
dc.subject.meshHumans
dc.subject.meshIncidence
dc.subject.meshInfant
dc.subject.meshMale
dc.subject.meshMedulloblastoma
dc.titleThe incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma.en
dc.typeArticleen
dc.contributor.departmentCRC Department of Cancer Genetics, Paterson Institute for Cancer Research, Manchester, UK.en
dc.identifier.journalBritish Journal of Canceren
html.description.abstractWe 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.


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