• Journal of the Korean Society of Radiology
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• v.82 no.5
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• pp.1346-1351
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• 2021

Mazabraud syndrome is a rare benign disease that is accompanied by polyostotic fibrous dysplasia and intramuscular myxoma. Malignant transformation of fibrous dysplasia occurs in approximately 1% of cases. To date, only eight cases of malignant transformation, of fibrous dysplasia to osteosarcoma, in Mazabraud syndrome have been reported worldwide. The authors report the first case of osteosarcomatous transformation in a patient with Mazabraud syndrome in the Republic of Korea, focusing on imaging findings.