We report the case of a 16-year-old boy who presented with mesenchymal chondrosarcoma involving the ethmoid sinus, right orbit, nasal cavity, and anterior cranial fossa. The tumor was totally resected by craniofacial surgery; the patient's postoperative course was uneventful. Microscopically, the tumor was composed of highly cellular undifferentiated small cells, alternating with zones of cartilaginous tissues. Mesenchymal chondrosarcoma is a rare, aggressive variant of chondrosarcoma, which might be encountered more frequently due to the advances in neuroimaging and surgical procedures.
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