397 Pulmonary Artery Intimal Sarcoma Presenting With Right Ventricular Thrombus

Abstract

Sarcomas as a whole comprise less than 1% of all malignancies. The first intimal sarcoma of the pulmonary artery was described in the literature in 1923 with less than 300 cases reported in the English literature since. The current incidence is estimated to be between 0.001% to 0.003%. We present the case of a 70-year-old male who presented with a one-day history of right sided pleuritic chest pain, dyspnoea, bilateral lower limb oedema and tachycardia. There was a clinical suspicion of a pulmonary embolism due to the history of pleuritic chest discomfort, moderate Wells score and electrocardiogram (ECG) changes. A computed tomography pulmonary angiogram (CTPA) performed showed a large thrombus in the right ventricle extending into the pulmonary trunk with features of right heart strain. The difficulty in diagnosis of pulmonary artery intimal sarcoma lies within the pathology of the presentation of patients. Pulmonary thrombus versus sarcoma is diagnostically very challenging. Patients frequently present with pulmonary thromboembolism, pulmonary trunk aneurysm and cardiopulmonary arrest. Radiologically filling defects itself on CTPA further leads to misdiagnosis. In this case, the diagnostic challenge was increased for the pathologists given the large amount of fibrin clot and necrotic tissue on histology. Based on a literature review this is the first reported case of a pulmonary artery sarcoma with raised D-dimer due to admixed/overlying fibrin tissue.