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Dedifferentiated parosteal osteosarcoma is an uncommon variant of osteosarcoma. Dedifferentiation is known to be associated with a greater metastatic potential and a more rapid lethal clinical course. Thus recognition of dedifferentiation is important to establish the treatment strategy. But there m...

Dedifferentiated parosteal osteosarcoma is an uncommon variant of osteosarcoma. Dedifferentiation is known to be associated with a greater metastatic potential and a more rapid lethal clinical course. Thus recognition of dedifferentiation is important to establish the treatment strategy. But there may be few significant clinical clues to distinguish between dedifferentiated parosteal osteosarcoma and conventional one. A 29-year-old woman presented with 2-year history of discomfort and swelling in her proximal thigh. Examination showed a large, hard, non-mobile mass. Radiographs revealed a large ossified mass attached to the proximal femur. Diagnosis of parosteal osteosarcoma was established by MRI and needle biopsy. But she had a history of abrupt severe thigh pain and increased swelling before surgery. Follow up MRI showed enlargement of mass with invasion to muscle around tumor. The patient underwent an en-bloc resection of tumor and reconstruction. Histological examination showed parosteal sarcoma with dedifferentiation. The patient expired due to local recurrence of tumor and distant lung metastasis 2 months after the surgery. In case with rapid growth of a lesion or unusual severe pain, one must have a high index of suspicion with regard to dedifferentiation.

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