Background: Osteosarcoma of the jaw (JOS) constituting 5% to 13% of all osteosarcoma is a locally aggressive malignant mesenchymal tumor with high tendency to local recurrence and the ability to produce tumor osteoid. Compared to osteosarcoma of the remaining skeleton (SOS) JOS metastasizes relatively rarely and relatively late. It differs significantly from SOS in its biological behaviour.
Case report: The presented case deals with a 60-year-old male patient suffering from a newly occurred polypous mucosal tumor of the right-sided maxillary alveolar ridge bioptically diagnosed as a granulation tissue polyp first. After transmaxillary resection of the tumor and consultation of a bone tumor reference center the final diagnosis of a high-grade chondroblastic osteosarcoma could be made. The recommended radical resection of the tumor was declined by the patient because of the expected cosmetic consequences. After surgical reduction of the rapidly growing sarcoma chemotherapy according to the COOS/EURO-B.O.S.S. protocol was initiated at a stage when computed tomography showed diffuse metastatic disease to the lungs. Chemotherapy could delay the progress of the disease only temporarily. The patient died from respiratory insufficiency.
Conclusion: In JOS radical surgical resection is the therapy of first choice. For substantiation of the diagnosis and central registration of the cases a bone tumor reference center should be contacted.