Primary hepatic leiomyosarcoma (LMS) is a rare malignancy of the liver in adults, with variable overall survival (OS). The main purpose of this study was to identify prognostic factors of improved survival. Relevant data were abstracted from MEDLINE, EMBASE, and Academic Search Premiere (ASP), and from the medical records of a patient treated at our institution. Multivariate analysis was conducted by generating Cox proportional hazard ratios. We identified 109 patients with primary hepatic LMS; 95 were included for basic description, and 84 for OS analysis. Median age was 58 years (range 19–86 years), 45.0% of the patients were female, and 21.1% were AJCC stage IV. The primary treatment for all 95 patients was as follows: 61.1% had surgery, 13.7% had chemotherapy, 5.3% had liver transplantation, 2.1% received radiotherapy, and 14.7% were managed conservatively. Median OS was 19 months (range 0–181 months) with 1-, 2-, and 5-year survival rates of 61.2, 41.1, and 14.5%, respectively. In a multivariate analysis, smaller size of the largest hepatic lesion (p = 0.038) and negative resection margin (p = 0.0004) were associated with improved survival. Complete surgical resection with clear margin is necessary to improve survival of patients with primary hepatic LMS.

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