| Summary: | Pulmonary artery intimal sarcoma (PAIS) is a rare and highly malignant neoplasm that presents similarly to pulmonary embolism. Due to the rarity of presentation and high mortality, there is limited data on its heterogeneous presentation, epidemiology, and treatment. We present a case of pulmonary artery intimal sarcoma mimicking features of pneumonia and pulmonary embolism. Our patient was a 43-year-old non-smoker male with no past medical history who presented with a cough with hemoptysis. Initial CT imaging showed acute right pulmonary embolism with right lung pulmonary infarct. The patient was treated with anticoagulation. On follow-up, he continued to complain of hemoptysis. Repeat CT lung now showed a moderate-sized right hilar mass. Endobronchial ultrasound-guided fine needle aspiration biopsy of the hilar mass with bronchial lavage showed malignant cells with immunohistochemistry positive for vimentin. The patient underwent a right pneumonectomy when he was later diagnosed with pulmonary artery intimal sarcoma. He was subsequently started on epirubicin, ifosfamide, and mesna and is currently on 3-monthly CT surveillance with no disease recurrence.
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