Abstract: Objective To investigate clinicopathologic characteristics, diagnosis and differential diagnosis and prognosis of perivascular epithelioid cell tumor (PEComa) of liver.Methods The clinical and pathological features of 3 cases with liver PEComa were analyzed by histopathological and immunohistochemical testing. Clinical and follow-up information was obtained. The published relevant literatures were reviewed.Results All 3 patients were females, aged from 39 to 60 years. The most common symptoms were cough with sputum and exertional dyspnea; 2 case was asymptomatic and 1 case with bellyache. Microscopically, the boundary between tumor and normal liver tissue was clear, the epithelioid tumor cells were solid lamellar or nested arrangement, the cytoplasm was eosinophilic or translucent, abundant vascular network and fat vacuoles were found in the stroma. Immunohistochemically, the tumor cells were positive for Vim, HMB45, Melan-A and SMA. The average follow-up was 37 months, no tumor recurrence or metastasis was found.Conclusion PEComa of liver is rare and the clinical symptoms are atypical, definite diagnosis depends on characteristic histological morphology and immunohistochemical findings.

Key words: Perivascular epithelioid cell tumor, Liver neoplasma, Clinicopathologic