Epithelioid osteosarcoma (OS) is a rare sub-type of OS with an aggressive behavior. An epithelioid OS was diagnosed in an 8-year-old female with painful swelling of the left jaw. After two courses of chemotherapy (cisplatin/methotrexate/doxorubicin), the patient presented a progressive disease. After hemimandibulectomy, 13 courses of post-operative chemotherapy (cisplatin/methotrexate/doxorubicin/ifosfamide) were performed. Histological and ultra-structural examination showed a high grade neoplasm consisting of sheets of epithelioid cells with focal osteoid formation. The patient is alive and in complete remission 42 months from diagnosis.
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