Abstract
Gnathic osteosarcoma (OS), which includes mandibular and maxillary jaw OSs, account for 6%-9% of OS. Single-institutional and multi-institutional retrospective studies, as well as population-level databases, suggest that clinical differences exist among gnathic OS and OS of other sides, including other craniofacial OS. To date, no specific prospective studies of gnathic OS have been reported, and aspects of clinical management are controversial. Some elements of care are aligned with extragnathic OS, e.g., margin negative (R0) surgery, whereas others, such as chemotherapy and radiation, are not clearly defined. The authors reviewed the available literature for the diagnosis, treatment, and supportive/survivorship care patients with gnathic OS and offer consensus statements for the comprehensive management of this rare disease.