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Treatment: Localized disease is treated with neoadjuvant chemotherapy, radiation therapy, and surgical resetion with wide margins. For those with metastatic disease, the primary form of therapy is systemic chemotherapy. Cases that resemble Ewing sarcoma respond better to chemotherapy.

Prognosis: Mesenchymal chondrosarcoma (MC) is an aggressive malignancy with a dismal prognosis; however, it has a variable clinical course that cannot be predicted by histologic features. Some patients progress rapidly and succumb to the disease within a few months after diagnosis. Others have a prolonged course and survive for years after initial surgery.

The median survival is about 38 months. The 5-yr and 10-yr survival rates are 55% and 28% respectively. Tumors of mandible have a better prognosis with 5-yr and 10-yr survival rates of 82% and 56% respectively. Metastases occur to the lung (most commonly), regional lymph nodes, other bones, and viscera and can appear several years after the initial diagnosis.

This example of MC was composed predominantly of cartilaginous areas with only small foci of undifferentiated mesenchymal cells squeezed in between cartilaginous nodules.

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