WINSLOW H. ARNOLD, M.D.
Three of six siblings developed the uncommon tumor medullary fibrosarcoma of bone. An uncle died of a metastatic bone tumor. The siblings and 5 (possibly 11) of their kindred had an underlying radiologic abnormality in the diaphyses of the large tubular bones. In two instances this was shown to be caused by areas of marrow necrosis, indistinguishable from bone infarcts. The bone lesions appeared in the kindred in a pattern consistent with the inheritance of an autosomal dominant trait. The mechanism of action of the abnormal gene was not found. The previously suspected association between healing bone infarcts and medullary fibrosarcoma is reemphasized.
ARNOLD WH. Hereditary Bone Dysplasia with Sarcomatous Degeneration: Study of a Family. Ann Intern Med. 1973;78:902–906. doi: 10.7326/0003-4819-78-6-902
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Published: Ann Intern Med. 1973;78(6):902-906.
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Print ISSN: 0003-4819 | Online ISSN: 1539-3704
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