Primary sarcomas of the aorta, pulmonary artery, superior vena cava, inferior vena cava, and the heart are rare neoplasms. Aortic sarcomas are broadly categorized as either primarily luminal or primarily mural, with luminal sarcomas more likely to be misdiagnosed as thrombus. Pulmonary artery sarcomas are often mistaken for pulmonary embolism both clinically and at imaging. Vena caval sarcomas appear as intraluminal or extraluminal masses connecting to or filling the veins. The most common are leiomyosarcomas of the inferior vena cava. Primary sarcomas of the heart are rare and usually appear as heterogeneous aggressive masses.
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