The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma.
Evans, D Gareth R Farndon, P A Burnell, L D Gattamaneni, Rao Birch, Jillian M
Evans, D Gareth R
Farndon, P A
Burnell, L D
Gattamaneni, Rao
Birch, Jillian M
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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.
Authors
Evans, D Gareth R
Farndon, P A
Burnell, L D
Gattamaneni, Rao
Birch, Jillian M
Farndon, P A
Burnell, L D
Gattamaneni, Rao
Birch, Jillian M
Affiliation
CRC Department of Cancer Genetics, Paterson Institute for Cancer Research, Manchester, UK.
Description
Date
1991-11
Publisher
Keywords
Cerebellar Cancer
Type
Article
Citation
The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma. 1991, 64 (5):959-61 Br. J. Cancer