Osteopathia Striata with Concomitant Fibroblastic Osteosarcoma of the Femur. A Potential Malignant Risk

We report a case of a 33-year old female with an unusual presentation of high-grade osteosarcoma superimposed on osteopathia striata. Few cases of malignancy arising in sclerosing bone dysplasia have been reported in literature. Of the few reported ones, osteosarcoma was associated with melorheostosis in two cases, with osteopoikilosis in the one case, and with combined melorheostosis and osteopathia striata in one case. To the best of our knowledge the current case is the first one of a high-grade osteosarcoma arising from isolated osteopathia striata, not combined with other forms of sclerosing bone dysplasia.