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J Med Genet. 1981 Dec;18(6):428-30.

Hereditary multiple exostoses: report of a kindred.

Abstract

In a large family with 37 members with multiple exostoses, only one person has developed sarcomatous degeneration of a lesion. Our review of published reports revealed great variation in the incidence of malignancy in multiple exostoses (10 to 25%). Most studies had sampling errors leading to the apparent overstatement of risk. In large pedigrees with essentially complete ascertainment of affected subjects, the risk of malignancy is nearer 3% or less. This lower risk for malignancy may be more appropriate in counselling affected subjects.

PMID:
6977644
[PubMed - indexed for MEDLINE]
PMCID:
PMC1048787
Free PMC Article
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