Primary pulmonary artery sarcomas are rare tumors that are frequently misdiagnosed as chronic pulmonary emboli. We present classic imaging findings and review data from 136 previously reported sarcomas. We believe that the imaging findings can be quite specific, especially when the disease is advanced. Despite a recent increase in antemortem diagnosis, the prognosis remains poor. Surgery prolongs survival time and is potentially curative. Experience with adjuvant chemotherapy and radiotherapy is limited.

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