期刊
JOURNAL OF BONE AND JOINT SURGERY-BRITISH VOLUME
卷 91B, 期 9, 页码 1223-1226出版社
BRITISH EDITORIAL SOC BONE JOINT SURGERY
DOI: 10.1302/0301-620X.91B9.22299
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We identified eight patients of 2900 with a primary malignant bone tumour who had coexisting neurofibromatosis type 1. This was a much higher incidence than would be expected by chance. The patients had a mean age of 22.4 years ( 9 to 54): five were male. Two patients subsequently developed a second bone sarcoma, one of which was radiation induced. Four of the primary tumours were osteosarcomas, four were spindle-cell sarcomas and one a Ewing's sarcoma. All the patients were treated with chemotherapy and surgery: six of the eight appear to be cured. This study suggests a possible relationship between neurofibromatosis type 1 and the development of a bone sarcoma, the increased risk being estimated at eight times that of the normal population. We recommend that further research into this possible link should be considered.
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