The primary pulmonary artery sarcoma is a rare disease and pulmonary thromboembolism must be considered for differential diagnosis. In the CT scan a low-attenuation filling defect occupying the entire luminal diameter of the pulmonary artery, expansion of the involved arteries or extraluminal tumor extension can lead to a suspected diagnosis. Furthermore in case of a progression of the findings under sufficient anticoagulant therapy a primary pulmonary sarcoma has to be considered as differential diagnosis. Complete surgical resection offers the only chance of cure.
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