Abstract: Objective This paper summarized the clinical features of 5 patients with hepatic perivascular epithelioid cell tumor (PEComa) in order to improve the clinician’s ability for diagnosis and differentiation of intrahepatic occupying lesions. Methods The clinical materials of 5 patients with PEComa were retrospectively analyzed with the emphasis on imaging features. All patients underwent gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid (Gd-EOB-DTPA) MRI scan and hepatectomy. Results Serum bilirubin levels increased in 2 cases, ALT increased in 2 cases and AST increased in 1 case with normal serum AFP, albumin and prothrombin time in all 5 patients; serum HBsAg positive in 3 cases; all the patients had a single intrahepatic lesion, with in the right lobe in 4 cases and in the left in 1 cases; the tumor sizes were 1.2 ×1.3 cm-6.9×9.0 cm shaped lobulated or elliptical, with clear edges in 4 lesions and obscure edge in 1 lesion; the MRI T1WI showed low signal in 5 cases, T2WI showed high signal in 4 cases, intensified in artery phase in 5 cases, presenting as low signals in portal phase in 4 case, equal signal in 1 case, and DWI showed high signal in 5 cases; all 5 patients got along well as followed-up for 10-263 months. Conclusion The PEComa could have similar clinical and imaging manifestations to primary liver cancer, but might have a good prognosis. The clinicians should paid attention to differentiation when the patients have no background of viral hepatitis or cirrhosis, especially when there is a uniform enhancement in arterial phase accompanied by intratumoral vessels or early venous drainage, high signal at tumor edge in portal vein phase, and some signs of fat infiltration.

Key words: Hepatoma, Hepatic perivascular epithelioid cell tumor, Magnetic resonance imaging, Diagnosis, Clinical feature