Abstract
This case describes a 5-month-old male who presented with respiratory distress and superior vena cava syndrome due to malignant ectomesenchymoma (MEM). Surgical treatment was limited to biopsy because of the extensive tumor involvement of the heart and great vessels. The patient was treated with sarcoma-directed chemotherapy. This is the first reported case of a primary MEM in the chest as well as the first case of MEM with cardiac invasion. The work up, differential diagnosis, and treatment of this extremely rare entity are presented.
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