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

2.50
Hdl Handle:
http://hdl.handle.net/10541/108463
Title:
The incidence of Gorlin syndrome in 173 consecutive cases of medulloblastoma.
Authors:
Evans, D Gareth R; Farndon, P A; Burnell, L D; Gattamaneni, Rao; Birch, Jillian M
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.
Affiliation:
CRC Department of Cancer Genetics, Paterson Institute for Cancer Research, Manchester, UK.
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
Issue Date:
Nov-1991
URI:
http://hdl.handle.net/10541/108463
PubMed ID:
1931625
Type:
Article
Language:
en
ISSN:
0007-0920
Appears in Collections:
All Christie Publications ; All Paterson Institute for Cancer Research

Full metadata record

DC FieldValue Language
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.en
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

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