Primary pulmonary artery sarcoma resembling chronic thromboembolic pulmonary disease

Two cases of primary pulmonary artery sarcoma resembling chronic thromboemb olic disease features are presented. Tumour identification was achieved aft er pulmonary thromboendarterectomy; which was indicated by documentation of a prothrombotic state in both patients. A doubtful history of pulmonary emboli or deep venous thrombosis should ale rt medical personnel to the possible presence of a primary pulmonary artery sarcoma. Advanced imaging methods such as gadolinium-enhanced magnetic res onance imaging could be useful in considering pulmonary thromboendarterecto my. If a tumour is detected, its surgical resection should be considered with c aution, taking into account the poor survival results. Invasion of the adve ntitia or the right ventricle, as documented in the present cases, is unusu al. As far as the present authors know, this is the first report of this kind o f tumour and its coexistence with an activated protein C resistance state a nd type II heparin-induced thrombocytopenia.