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J Med Genet. 1981 December; 18(6): 428–430.
PMCID: PMC1048787

Hereditary multiple exostoses: report of a kindred.


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.

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Selected References

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  • KNIGHT JD. Sarcomatous change in three brothers with diaphysial aclasis. Br Med J. 1960 Apr 2;1(5178):1013–1015. [PMC free article] [PubMed]
  • SOLOMON L. HEREDITARY MULTIPLE EXOSTOSIS. Am J Hum Genet. 1964 Sep;16:351–363. [PubMed]
  • Solomon L. Chondrosarcoma in hereditary multiple exostosis. S Afr Med J. 1974 Apr 6;48(16):671–676. [PubMed]

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