ABSTRACT
BACKGROUND: Primary pulmonary artery sarcomas are rare malignant vascular tumors and carry a very poor prognosis. Due to overlapping clinical and radiological features, the differentiation between pulmonary artery thromboembolism and pulmonary artery sarcoma can be challenging. CASE PRESENTATION: We herein present clinical, radiological and pathological features of primary pulmonary artery high grade sarcoma (angiosarcoma) in a 59-year-old male. The patient presented with a history of breathlessness on exertion of 2-months duration and was misdiagnosed as massive pulmonary thromboembolism on initial CT imaging. CONCLUSION: Great similarity with significant degree of overlap in clinical and radiologic presentation makes differentiation of pulmonary artery sarcomas and thromboembolism a diagnostic challenge. Even though they are exceptionally rare, one should always consider it as differential diagnosis especially in cases with atypical clinical or imaging presentation.