Primary sarcoma of the pulmonary artery (PSPA) is extremely rare. Many cases are misdiagnosed as pulmonary arterial hypertension (PAH) because of chronic thromboembolic disease (CTD). Four cases of PSPA with the initial misdiagnosis are reported. The presence of a unique mass in the main pulmonary artery or proximal branches, rapidly progressive dyspnea, and constitutional symptoms should raise the suspicion of PSPA. The pathological diagnosis is usually confirmed during surgery, which is done along with adjuvant chemotherapy, the treatment of choice.
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