The ossifying fibromyxoid tumor (OFMT) of soft parts is a rare soft tissue neoplasm of uncertain lineage. The most common metastases are found in the lung. Herein, we present the first case report of pulmonary metastasis of ossifying fibromyxoid tumor with intracardiac extension, which was resected carefully using cardiopulmonary bypass and cardiac arrest. Subsequently pulmonary left lower lobectomy was performed. The patient recovered uneventfully and was discharged in after a few days. Recently, OFMT has been considered as a tumor of intermediate malignancy. We recommend wide surgical excision of primary tumor and radiotherapy. Early follow-up is mandatory.
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