Hepatic monotypic epithelioid angiomyolipoma with concomitant hepatocellular carcinoma

Hepatic monotypic epithelioid angiomyolipoma (AML) is a rare lesion in which the predominant population of an epithelioid component can mimic hepatocellular carcinoma (HCC). The hepatic epithelioid AML with concomitant HCC is extremely uncommon. In this study, we present the clinical and pathologic features of a case of hepatic monotypic epithelioid AML with concomitant HCC in a 63-year-old man. Imaging examinations revealed two masses located in the liver, measuring 83×63 mm and 37×27 mm separately, which exhibited an early contrast enhancement and a rapid washout on enhanced computed tomography (CT), so that HCC with intrahepatic metastases was suspected. The small tumor was removed for intraoperative frozen section examination. Grossly, the tumor was solitary, well-circumscribed, and non-encapsulated. Microscopically, it was composed purely of a trabecular arrangement of epithelioid cells with a sinusoidal pattern. Immunohistochemically, it was positive for HMB45, Melan-A, and alpha smooth muscle actin (α-SMA). Interestingly, the large tumor has the histologic features similar to those of the small one. However, it was positive for epithelioid markers and negative for the melanocytic markers. It reminds us that there is a possibility of coexistence of HAML and HCC in the liver. We believe that this might be the first case report of a hepatic monotypic epithelioid AML with concomitant HCC. The patient gave up treatment and died in 6 months after the operation in the follow-up.