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.