Cause analysis of mistreatment and misdiagnosis of primary pulmonary artery sarcoma

Abstract

Objective To improve the understanding of primary pulmonary artery sarcoma (PAS) and reduce the rate of misdiagnosis and mistreatment. Methods The clinical data of four PAS patients confirmed with biopsy and relevant foreign literatures were analysed to explore the causes of mistreatment and misdiagnosis of PAS. Results The clinical manifestations of PAS were similar to those of pulmonary thromboembolism,mainly including exertional dyspnea/short breath, chest distress, chest pain, cough, hemoptysis,syncope, etc. , without specificity. Color Doppler echocardiography showed right ventricular enlargement, tricuspid regurgitation, pulmonary arterial hypertension and clump like abnormal echo in right ventricular outflow tract and main pulmonary artery. Doppler vascular ultrasound showed lower extremity venous was normal. CT pulmonary angiography showed expansion of pulmonary artery, luminal stenosis,and large filling defect in main pulmonary artery and left/right pulmonary artery. Four cases were all misdiagnosed as pulmonary embolism before surgery,received thrombolytic and anticoagulant therapy, but the effect was poor. One case died and three cases relieved after operation, no one case received adjuvant chemotherapy and (or) radiotherapy. Conclusions PAS is rare in clinic, and is often misdiagnosed as pulmonary thromboembolism for thrombolytic anticoagulant therapy. The confirmed diagnosis needs biopsy. Comprehensive treatment based on surgery is effective for the disease. Key words: Sarcoma; Pulmonary artery; Pulmonary embolism; Misdiagnosis; Mistreatment