Hereditary multiple exostoses: report of a kindred.

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Abstract
In a large family with 37 members with multiple exostoses, only 1 person has developed sarcomatous degeneration of a lesion. A review of published reports revealed great variation in the incidence of malignancy in multiple exostoses (10-25%). 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 counseling affected subjects.

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