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Abstract Pulmonary artery intimal sarcoma (PAIS) is a rare malignancy which closely mimics acute or chronic pulmonary thromboembolism. There are clinical and radiological characteristics which may raise suspicion of this important differential diagnosis. These include disproportionately low d‐dimer, troponin T or NT‐proBNP, as well as characteristic findings on CT pulmonary angiography such as the ‘wall eclipsing sign’ and an non‐dependent position of filling defects in the large arteries. Prompt diagnosis avoids inappropriate anticoagulation and facilitates early surgical management which may improve prognosis. There is emerging evidence of an effective treatment paradigm with surgical resection and adjuvant chemotherapy. We present two cases of PAIS diagnosed at a single centre within a 2‐year period. We review the literature and demonstrate the features at presentation in our cases which were suggestive of the diagnosis.