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Very few cases of cervical, thoracic wall, mediastinum and cardiac primary myxofibrosarcoma have been documented, and to the best of our knowledge, only one primary pulmonary myxofibrosarcoma case has previously been reported in the English literature. Here, we present a case of primary pulmonary myxofibrosarcoma that extended to the left atrium through the pulmonary vein. In such rare clinical presentations, intracardiac tumor resection should precede pulmonary resection due to the possibility of catastrophic tumor embolisms.
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