探讨肝脏血管周上皮样细胞瘤（PEComa）的临床特点、诊治原则和预后。

回顾性分析2013年1月至2019年7月于中国医科大学附属盛京医院手术治疗且术后病理证实为肝脏PEComa的23例患者临床资料，结合文献讨论肝脏PEComa的临床特点、诊断情况、治疗及预后。

23例患者中女16例，男7例，中位年龄为50岁；肿瘤直径4.6（1.0~22.5）cm，以单发为主（87.0%），8例位于左半肝，11例位于右半肝，1例包含左、右半肝，1例位于尾状叶，1例肝、肾多发，1例肝、腹膜后多发，术前仅4例诊断为PEComa。Melan-A、HMB-45和SMA阳性率分别为92.9%（13/14）、85.7%（12/14）和76.9%（10/13）。所有患者术后均恢复良好后出院，随访期内未见复发。

肝脏PEComa是一种相对罕见的肝脏肿瘤，多为良性，影像学表现有一定特征性，但依赖于术后病理学和免疫组织化学检查确诊。术前能够确诊的良性PEComa可定期复查或保守治疗，手术切除预后较好。

To explore the clinical characteristics, treatment and diagnosis principle, and the prognosis of perivascular epithelioid cell tumor (PEComa) of the liver.

The clinical data of twenty-three patients with liver PEComa diagnosed by postoperative pathology in Shengjing Hospital of China Medical University from January 2013 to July 2019 were analyzed retrospectively. The clinical features, diagnosis, treatment and prognosis of liver PEComa were analyzed.

The median age of twenty-three patients was 50 years old, including sixteen females and seven males. The median diameter of the tumor was 4.6 (1.0-22.5) cm. Most tumors were solitary (87.0%). There were eight tumors located in the left lobe, eleven tumors in the right lobe, one tumor in both the left and right lobes, one tumor in the caudate lobe, one case in the liver and kidney, and one case in the retroperitoneum and the liver. Only four cases were diagnosed as PEComa before operation. The positive rates of Melan-A, HMB-45 and smooth muscle actin (SMA) by immunohistochemistry were 92.9% (13/14), 85.7% (12/14), and 76.9% (10/13) respectively. All patients received surgical treatment and were discharged safely after operation. No recurrence was found during the follow-up period.

Liver PEComa is a relatively rare liver tumor, which is mostly benign. There are some characteristics in imaging, but the diagnosis relies on postoperative pathology and immunohistochemistry. The benign PEComa can be diagnosed before operation and reexamined regularly or treated conservatively. The prognosis of surgical resection is good.