Primary chondromatous osteosarcoma of the pulmonary artery

Primary pulmonary sarcoma is an extremely rare malignancy, pulmonary osteogenic sarcoma is even more rare and has a poor prognosis. We present a report of a 58-year-old woman with primary pulmonary osteosarcoma occupying the right main pulmonary artery and extending to the main pulmonary artery. Right pneumonectomy was performed with total right main pulmonary artery resection under cardiopulmonary bypass. Microscopic examination demonstrated that tumor cells contained mainly chondromatous components and were atypical cells that became osteoid. There is no sign of recurrence 24 months postoperatively. The present case is the only case yet reported with even an intermediate-term survival without recurrence.