Clinical presentation and surgical treatment of primary pulmonary artery sarcoma

作者：Long Deng, Jiade Zhu, Jianping Xu, Shaoxian Guo, Sheng Liu, Yunhu Song · 发表于：Interactive Cardiovascular and Thoracic Surgery · 年份：2017 · DOI：10.1093/icvts/ivx318 · 被引用次数：53 · 研究领域：Cardiac tumors and thrombi、Sarcoma Diagnosis and Treatment、Lung Cancer Diagnosis and Treatment

OBJECTIVES: Pulmonary artery sarcoma (PAS) is a rare and devastating disease. The diagnosis modality and optimal surgical strategy remain unclear. The aim of this study was to report our surgical experience in this disease. METHODS: Between 2000 and 2017, 13 patients underwent operations for PAS at our institution. Five patients underwent tumour resection alone, whereas the other 8 patients received pulmonary endarterectomy. The medical records were retrospectively reviewed to evaluate the clinical characteristics, operative findings and the postoperative outcomes. RESULTS: There were 2 perioperative deaths, both had unresolved severe pulmonary hypertension. Follow-up was completed for all patients with a mean duration of 14.3 months (1-48 months). During follow-up, 6 patients died after a mean survival time of 20.2 months (4-48 months). For the 5 patients alive at follow-up (mean duration of 7.2 months), 3 already had local recurrence. For all 13 patients, the cumulative survival rates at 1, 2 and 3 years were 56.4, 37.6 and 18.8%, respectively. The 8 patients treated with pulmonary endarterectomy seemed to have a better survival rate compared with the remaining 5 patients without endarterectomy, although the difference was not statistically significant. CONCLUSIONS: Primary PAS is often mistaken for chronic pulmonary thromboembolism. Surgical intervention is the mainstay of treatment for PAS, but the prognosis after surgery remains poor. Compared to isolated tumour resectio...