Complications of the naevoid basal cell carcinoma syndrome: results of a population based study.
Affiliation
Department of Medical Genetics, St Mary's Hospital, Manchester.Issue Date
1993-06
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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.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 GeneticsDOI
10.1136/jmg.30.6.460PubMed ID
8326488Type
ArticleLanguage
enISSN
0022-2593ae974a485f413a2113503eed53cd6c53
10.1136/jmg.30.6.460
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