Complications of the naevoid basal cell carcinoma syndrome: results of a population based study.

2.50
Hdl Handle:
http://hdl.handle.net/10541/100334
Title:
Complications of the naevoid basal cell carcinoma syndrome: results of a population based study.
Authors:
Evans, D Gareth R; Ladusans, E J; Rimmer, S; Burnell, L D; Thakker, N; Farndon, P A
Abstract:
There are many potential complications which have been reported in association with the naevoid basal cell carcinoma syndrome. We have been able to show the relative frequencies of these problems in a population based study of 84 cases in the north west of England. The major complications of basal cell carcinomas and jaw cysts occur in over 90% of patients by 40 years of age, but may both occur before 10 years of age. Less well described complications are ovarian calcification or fibroma (24%), medulloblastoma (5%), cardiac fibroma (3%), cleft palate (5%), and ophthalmic abnormalities such as squint or cataract (26%). This study more clearly defines the possible complications of the syndrome and gives clearer guidelines for counselling and screening affected and at risk persons.
Affiliation:
Department of Medical Genetics, St Mary's Hospital, Manchester.
Citation:
Complications of the naevoid basal cell carcinoma syndrome: results of a population based study. 1993, 30 (6):460-4 J. Med. Genet.
Journal:
Journal of Medical Genetics
Issue Date:
Jun-1993
URI:
http://hdl.handle.net/10541/100334
DOI:
10.1136/jmg.30.6.460
PubMed ID:
8326488
Type:
Article
Language:
en
ISSN:
0022-2593
Appears in Collections:
All Paterson Institute for Cancer Research

Full metadata record

DC FieldValue Language
dc.contributor.authorEvans, D Gareth Ren
dc.contributor.authorLadusans, E Jen
dc.contributor.authorRimmer, Sen
dc.contributor.authorBurnell, L Den
dc.contributor.authorThakker, Nen
dc.contributor.authorFarndon, P Aen
dc.date.accessioned2010-06-05T10:49:06Z-
dc.date.available2010-06-05T10:49:06Z-
dc.date.issued1993-06-
dc.identifier.citationComplications of the naevoid basal cell carcinoma syndrome: results of a population based study. 1993, 30 (6):460-4 J. Med. Genet.en
dc.identifier.issn0022-2593-
dc.identifier.pmid8326488-
dc.identifier.doi10.1136/jmg.30.6.460-
dc.identifier.urihttp://hdl.handle.net/10541/100334-
dc.description.abstractThere are many potential complications which have been reported in association with the naevoid basal cell carcinoma syndrome. We have been able to show the relative frequencies of these problems in a population based study of 84 cases in the north west of England. The major complications of basal cell carcinomas and jaw cysts occur in over 90% of patients by 40 years of age, but may both occur before 10 years of age. Less well described complications are ovarian calcification or fibroma (24%), medulloblastoma (5%), cardiac fibroma (3%), cleft palate (5%), and ophthalmic abnormalities such as squint or cataract (26%). This study more clearly defines the possible complications of the syndrome and gives clearer guidelines for counselling and screening affected and at risk persons.en
dc.language.isoenen
dc.subjectBrain Canceren
dc.subjectHeart Canceren
dc.subjectOvarian Canceren
dc.subjectSkin Canceren
dc.subject.meshAdolescent-
dc.subject.meshAdult-
dc.subject.meshBasal Cell Nevus Syndrome-
dc.subject.meshBrain Neoplasms-
dc.subject.meshBronchogenic Cyst-
dc.subject.meshCarcinoma, Basal Cell-
dc.subject.meshChild-
dc.subject.meshChild, Preschool-
dc.subject.meshCleft Lip-
dc.subject.meshCleft Palate-
dc.subject.meshEngland-
dc.subject.meshEye Diseases-
dc.subject.meshFemale-
dc.subject.meshFollow-Up Studies-
dc.subject.meshHeart Neoplasms-
dc.subject.meshHodgkin Disease-
dc.subject.meshHumans-
dc.subject.meshInfant-
dc.subject.meshJaw Cysts-
dc.subject.meshMedulloblastoma-
dc.subject.meshOvarian Diseases-
dc.subject.meshOvarian Neoplasms-
dc.subject.meshPrevalence-
dc.subject.meshSkin Neoplasms-
dc.titleComplications of the naevoid basal cell carcinoma syndrome: results of a population based study.en
dc.typeArticleen
dc.contributor.departmentDepartment of Medical Genetics, St Mary's Hospital, Manchester.en
dc.identifier.journalJournal of Medical Geneticsen

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