Repeated dedifferentiation of low-grade intraosseous osteosarcoma

Low-grade intraosseous osteosarcoma is an uncommon bone tumor that is characterized by minimum cytological atypism and a much better prognosis than conventional osteosarcoma. This report describes a patient who had a low-grade osteosarcoma that mimicked fibrous dysplasia (FD). The tumor had an area of high-grade sarcoma at the initial diagnosis. Ten years after incomplete resection of FD-like tumor, local recurrence with areas of high-grade tumor developed. This case illustrates the potential of dedifferentiation in low-grade intraosseous osteosarcoma.