Osteosarcoma is the most common primary malignant neoplasm of the bone, with a reported incidence of 1 per 100 000, 6% to 10% of which present with a primary tumor of the head and neck.1More than 500 cases have been reported in the literature. The majority of head and neck osteosarcomas involve the mandible and the maxilla, and these tumors typically carry a better prognosis than those in extragnathic locations.2Primary occurrence at the base of the skull is rare.2The pathogenesis of osteosarcoma remains unknown. The association with Paget disease of bone has been well documented, and Kansara and Thomas3recently reviewed the molecular pathogenesis, with particular emphasis on the retinoblastoma suppressor gene and TP53 (tumor protein p53). Patel et al4reported osteosarcomas arising as sequelae from previous irradiation.
Pathology Quiz Case: Diagnosis. Arch Otolaryngol Head Neck Surg. 2008;134(12):1344. doi:10.1001/archotol.134.12.1344