[Primary sarcoma of A. pulmonalis: a rare cause of cor pulmonale]

A 64-year-old female presented with fever and weight loss, accompanied by normochromic normocytic anaemia with high erythrocyte sedimentation rate. She developed severe cor pulmonale. Ventilation-perfusion scintigraphy revealed a non-matched perfusion defect of the entire right lung with impaired perfusion on the left side. The CT scan of the thorax revealed an intravascular mass which completely blocked the right pulmonary artery with incomplete obstruction of the pulmonary trunk. Treatment with anticoagulants was started but failed. The presumably thrombotic mass was removed by thoracotomy; the histopathological diagnosis was: sarcoma of the pulmonary artery, incompletely resected. Postoperatively, the condition of the patient showed initial improvement, but she later developed fatal mediastinitis. Pulmonary artery sarcoma is a rare cause of cor pulmonale. Diagnosis is usually only established at operation, curative resection is uncommon and the prognosis is therefore poor--most patients die within months after the diagnosis has been made.