Epithelioid osteosarcoma of the mandible: a rare case with unusual immunoprofile

A 42-year-old woman presented a large, nontender, quickly progressive mass in the left mandible. Radiograph showed extensive destruction of the angle, posterior body, and ramus of the left side of the mandible. The patient was surgically treated by hemimandibulectomy. Microscopically, the tumor was composed of large epithelioid cells, many of them showing polarized nuclei, and evident eosinophilic cytoplasm. The predominant pattern was trabecular, and rosette-like structures were also observed. Typical osteoid-containing cells surrounded by malignant cells were found in a few areas. Immunohistochemistry for a large panel of antibodies showed positivity for osteocalcin, osteonectin, osteopontin, VS38c, and S-100. CD34 saliented the hemangiopericytoma-like distribution of the blood vessels. Collagen I was focally positive for the extracellular matrix and malignant osteoid. All other markers were negative, including vimentin and cytokeratins. To the best of our knowledge, this is the first case of epithelioid osteosarcoma affecting the mandible and the second case affecting the jaws that has been reported in the literature.