A 34-year-old man was admitted with a putative diagnosis of pulmonary artery trunk thromboembolism. Magnetic resonance imaging and angiography showed a solid mass extending from the pulmonary trunk to the right pulmonary artery. During surgery, a tumor was found arising from the pulmonary valve annulus. The tumor was completely removed and pulmonary valvuloplasty was performed. The result was satisfactory. Histopathology showed the tumor to be a leiomyosarcoma.
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