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The vast majority of adult primary cardiac tumours (75%) are benign. The differentiation between malignant and benign lesions based on imaging is often difficult. Furthermore, it is challenging to distinguish between a cardiac myxoma and a myxosarcoma histologically. We report the case of a 48-year-old female who underwent resection of myxoma. Fourteen months postoperatively, she developed dyspnoea and evidence of local recurrence was observed. An open biopsy was performed and compared with the initially resected specimen. A primary cardiac myxosarcoma was diagnosed. Extended resection of the tumour including a part of the left atrium and the left lung was performed. Follow-up at 4 years shows no radiological evidence of any further recurrence and the patient is satisfied with a good quality of life. Despite the infrequent nature and particularly in view of the poor prognosis of cardiac myxosarcoma with a median overall survival of ∼12-17 months, we were able to demonstrate in our case that, with an extensive medical and surgical therapy and an interdisciplinary approach, a long-term disease-free survival can be achieved.

Long-term survival after extended resection of primary atrial myxosarcoma