Abstract
A 34-year-old man presented with an expansile, erosive tumor involving the left side of the mandible, with secondary invasion into the maxilla, measuring 13 Χ 7 cm. Microscopic analysis revealed a malignant small round cell neoplasm with focal cartilaginous differentiation. Immunohistochemical analysis revealed positivity for vimentin, NSE and CD99 with primitive small round cells, and S100 positivity with neoplastic chondrocytes. To the best of our knowledge, this is perhaps the largest reported case of mesenchymal chondrosarcoma of the maxillofacial region. Diagnosed as inoperable, he was treated with radiation and chemotherapy only to die within a few months.
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