Primary pulmonary artery sarcoma resembling chronic thromboembolic pulmonary disease

Two cases of primary pulmonary artery sarcoma resembling chronic thromboembolic disease features are presented. Tumour identification was achieved after pulmonarv thromboendarterectomy, which was indicated by documentation of a prothrombotic state in both patients. A doubtful history of pulmonary emboli or deep venous thrombosis should alert medical personnel to the possible presence of a primary pulmonary artery sarcoma. Advanced imaging methods such as gadolinium-enhanced magnetic resonance imaging could be useful in considering pulmonary thromboendarterectomy. If a tumour is detected, its surgical resection should be considered with caution, taking into account the poor survival results. Invasion of the adventitia or the right ventricle, as documented in the present cases, is unusual. As far as the present authors know, this is the first report of this kind of tumour and its coexistence with an activated protein C resistance state and type II heparin-induced thrombocytopenia.