[Diagnosis and surgical treatment of pulmonary artery sarcoma]

To characterize the differential diagnostic characteristics and the surgical treatment efficacy of pulmonary artery sarcoma (PAS).

From November 2001 to January 2014, 19 PAS patients were diagnosed and 14 of them underwent surgery at Beijing Anzhen Hospital. And their data were retrospectively reviewed.

All 19 patients underwent pulmonary artery computed tomography angiography (CTA) scan. All showed a filling defect within the lumen of pulmonary artery with a sign of wall eclipsing. And 14 of them had pulmonary artery sarcoma confirmed through postoperative histopathological examination while another 5 patients were confirmed to have FDG abnormal high intake mass shadow on Positron emission tomography-computed tomography (PET-CT) scan. Fourteen patients underwent surgery, including pulmonary endarterectomy (n = 12) and pneumonectomy (n = 2), and another five had no indication for operation and died shortly. No perioperative death occurred for surgical patients. Five non-surgical patients survived (20.3 ± 11.2) days after discharge. And 14 postoperative patients survived (16.8 ± 3.8) months. The difference between two groups reached statistical significance (P = 0.000). The survival difference between two surgical procedures and between two pathological classifications did not reach statistical significance. Nine patients did not while another 5 received adjuvant radiotherapy and chemotherapy. Their average survivals were (12.3 ± 3.2) and (22.8 ± 4.3) months respectively. And the inter-group difference reached statistical significance (P = 0.000).

The sign of wall eclipsing on pulmonary artery CTA scan is pathognomonic for PAS. Radical surgical resection provides a longer survival than non-surgery and adjuvant chemotherapy may further extend survival.