Intimal sarcoma of the pulmonary artery is rare, but false diagnosis of this sarcoma as pulmonary embolism is not infrequent. The present study reports a case of pulmonary artery intimal sarcoma misdiagnosed as pulmonary artery embolism in a 37-year-old female patient. The patient was admitted to the cardiac intensive care unit of Guangdong General Hospital (Guangzhou, China) with the complaint of progressive exertional dyspnea over the past two years. Multi-slice spiral computed tomography, transthoracic echocardiography, right‑heart catheterization and cardiac magnetic resonance imaging revealed mimicking severe pulmonary embolism in the pulmonary trunk and right pulmonary artery, with symptoms including chest pain, cough and breathing difficulties. In addition, positron emission tomography-computed tomography results did not identify increased 18F‑fluorodeoxyglucose uptake and failed to distinguish whether the mass was a thrombus or a malignancy. The patient was diagnosed with pulmonary embolism and a subcutaneous injection of 5,000 AxaIU enoxaparin sodium (100 AXAIU/kg) was administered every 12 h, but no improvement was achieved after 5 days of treatment. Finally, pulmonary endarterectomy was performed to relieve the worsening clinical symptoms. The clinicopathological diagnosis was pulmonary artery intimal sarcoma with poor clinical outcome. For this type of tumor with fatal prognosis, early and correct diagnosis may lead to appropriate intervention and prolong survival.

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