Abstract

Abstract Primary pulmonary artery sarcomas are extremely rare tumors with approximately 400 cases reported so far in the literature. These patients present with a wide myriad of symptoms ranging from dyspnea to florid right heart failure, and the presentation is usually misdiagnosed and treated as chronic thromboembolic disease. Diagnostic imaging requires the use of multiple modalities including computed tomography, magnetic resonance imaging and echocardiography. In this E-challenge, we describe the case of a patient diagnosed preoperatively with pulmonic artery stenosis. Intraoperative imaging demonstrated a pulmonary artery lesion with subsequent biopsy and a pathological diagnosis.

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