Background. Primary pulmonary artery sarcomas are very rare and their histologic type, called leiomyosarcoma, is even rarer. Case Report. A 64-year-old woman presented with progressive weakness, fatigue, malaise, and dyspnea, and a marked elevation of pulmonary artery pressure was admitted. She was initially diagnosed with chronic pulmonary thromboembolism and chest computed tomography (CT) scan revealed that lobulated heterogeneous left hilar mass extended to precarinal and subcarinal space. MRI demonstrated a polypoid lesion at trunk with extension to left main pulmonary artery and its first branch. She was operated, a yellowish-shiny solid mass in pulmonary trunk was seen intraoperatively, and pulmonary endarterectomy was performed. Her tumor was pathologically diagnosed as pulmonary artery leiomyosarcoma. She died 3 months later after one chemotherapy course. Conclusion. Initially, the patient underwent surgery due to pulmonary embolism but, during the operation, the observed mass increased the probability of pulmonary artery neoplasm. Clinicians must consider pulmonary artery sarcoma when making the differential diagnosis for patients with pulmonary arteries masses. In addition to clinical prediction scores and CT and MRI findings to identify the patients with pulmonary artery sarcoma, PET scanning is the diagnosis of choice in differentiating embolism and neoplasm and is strongly recommended in these patients. 1. Introduction Primary pulmonary artery sarcomas are very rare and their histologic type called leiomyosarcoma is even rarer [1]. The incidence of primary pulmonary artery tumors is 0.001–0.03%, and they are nearly always highly malignant and typically obtain their origin from the intima [2]. The underlying pathophysiology of these tumors of the pulmonary arteries is still unclear [2, 3]. These tumors are frequently misdiagnosed as pulmonary thromboembolism in clinical settings. Many patients receive anticoagulant therapy without response, and many are diagnosed postmortem only [4]. Most of the tumors reported in the literature have involved the right ventricular outflow tract and the main pulmonary trunk, often extending into the main pulmonary artery branches [5]. 2. Case Report The patient was a 64-year-old woman presented with progressive generalized weakness and dyspnea for 6 months. She had no risk factors for thromboembolism. She had history of headache, weight loss, and nonproductive cough prior to the admission and one episode of presyncope last year. Her vital signs on admission were a temperature of 38°C, blood pressure of
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