The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma.
dc.contributor.author | Evans, D Gareth R | |
dc.contributor.author | Farndon, P A | |
dc.contributor.author | Burnell, L D | |
dc.contributor.author | Gattamaneni, Rao | |
dc.contributor.author | Birch, Jillian M | |
dc.date.accessioned | 2010-07-27T15:51:21Z | |
dc.date.available | 2010-07-27T15:51:21Z | |
dc.date.issued | 1991-11 | |
dc.identifier.citation | The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma. 1991, 64 (5):959-61 Br. J. Cancer | en |
dc.identifier.issn | 0007-0920 | |
dc.identifier.pmid | 1931625 | |
dc.identifier.uri | http://hdl.handle.net/10541/108463 | |
dc.description.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. | |
dc.language.iso | en | en |
dc.subject | Cerebellar Cancer | en |
dc.subject.mesh | Basal Cell Nevus Syndrome | |
dc.subject.mesh | Cerebellar Neoplasms | |
dc.subject.mesh | Child | |
dc.subject.mesh | Child, Preschool | |
dc.subject.mesh | Family Health | |
dc.subject.mesh | Female | |
dc.subject.mesh | Humans | |
dc.subject.mesh | Incidence | |
dc.subject.mesh | Infant | |
dc.subject.mesh | Male | |
dc.subject.mesh | Medulloblastoma | |
dc.title | The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma. | en |
dc.type | Article | en |
dc.contributor.department | CRC Department of Cancer Genetics, Paterson Institute for Cancer Research, Manchester, UK. | en |
dc.identifier.journal | British Journal of Cancer | en |
html.description.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. |