Primary pulmonary artery sarcomas (PPAS) are an extremely rare type of neoplasm (incidence of 0.001-0.03%). PPAS are malignant, aggressive and associated with poor survival outcomes1.
Factors that contribute to poor survival outcomes are:
1. Misdiagnosis: The similarity between the symptoms and radiological appearance of PPAS and the more common pathological processes of acute pulmonary embolism (APE) and chronic thromboembolic pulmonary hypertension (CTEPH) often result in misdiagnosis and incorrect treatment2.
2. Late presentation: The asymptomatic nature of the disease until vessel occlusion has commenced results in patients presenting with advanced-stage disease2.
In this case, a PPAS was diagnosed from the outset; however 47% of PPAS are misdiagnosed as APE3, which directly contributes to the poor survival outcomes characteristic of PPAS.
This case study aims to give the reader an understanding of how to distinguish a common APE from...
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