Aims and background: Primary myxofibrosarcoma of the heart is a rare malignancy. To the best of our knowledge, no more than 17 cases have been reported since 1963.
Methods and study design: We report the case of a 42-year-old man who presented with dyspnea and palpitation of one month's duration. Echocardiography and enhanced computed tomography revealed a large tumor in the left atrium.
Results: The patient underwent palliative excision of the tumor and histopathological analysis revealed it to be a low-grade myxofibrosarcoma. The patient developed a bone metastasis two years later and is still alive 26 months after the cardiac surgery.
Conclusions: Primary cardiac myxofibrosarcoma involves predominantly the left atrium and the most common symptom induced by the tumor is dyspnea. Disease outcome is often disappointing despite aggressive postoperative radiotherapy and chemotherapy.