Abstract
BACKGROUND: Pulmonary artery sarcoma is too rare for a randomized controlled trial or cohort study. We aimed to perform a retrospective analysis on the therapeutic effect of adjuvant therapy on postoperative survival time. METHODS: Medline and EMBASE database were searched for articles on pulmonary artery sarcoma published between 1923 and 2018. Age, gender, postoperative overall survival, therapeutic approach, tumor extension, tumor localization, status of resection margins, metastasis, surgical method were extracted as parameters to analyze postoperative overall survival. RESULTS: A total of 162 articles and 275 cases are included in analysis. Median postoperative overall survival time was 31 months. Patients who received adjuvant and/or neo-adjuvant therapy were associated with improved survival [hazard ratio (HR) =0.64, P=0.017, 95% confidence interval (CI): 0.45-0.92]. Patients with complete resection or without metastasis had longer postoperative overall survival compared with incomplete resection (HR =0.55, P=0.002, 95% CI: 0.37-0.79) or with metastasis (HR =6.01, P=0.000, 95% CI: 3.33-10.67). Subgroup analysis suggested chemotherapy was related with longer postoperative overall survival (HR =0.63, P=0.015, 95% CI: 0.43-0.91), especially for patients with incomplete resection (HR =0.53, P=0.025, 95% CI: 0.31-0.92) and metastasis (HR =0.44, P=0.000, 95% CI: 0.28-0.68). CONCLUSIONS: Radical surgery offers the only chance to cure pulmonary artery sarcoma. Palliative and aggressive surgery can relieve the symptoms and extend the life expectance. Patients can benefit from adjuvant and/or neo-adjuvant chemotherapy, especially those who have metastasis or undergo incomplete resection.