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a hepatologie

Gastroenterology and Hepatology

Gastroent Hepatol 2022; 76(2): 121–126. doi: 10.48095/ccgh2022121.

Liver transplantation for hepatic PEComa mimicking hepatocellular carcinoma

Jana Selucká1, Jiří Froněk  2, Luděk Voska  3, Dana Kautznerová  4, Pavel Taimr  1

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PEComas (perivascular epithelioid cell tumors) are rare mesenchymal tumours composed of epithelial perivascular cells. Most of these tumours have indolent behaviour, but occasionally they can have malignant potential. PEComas occur at any anatomical location and are 4 times more frequent in younger and middle-aged women. The diagnosis of PEComas is established incidentally in about 20% patients as a result of abdominal imaging. A typical microscopic feature of PEComas is the presence of perivascular epithelioid cells co-expressing both muscle and melanocytic markers. The only causal therapy is surgery. Our case report points at The diagnostic challenge of PEComas in, it describes a patient with malignant hepatic angiomyolipoma, who underwent liver transplantation for a bioptically proven diagnosis of hepatocellular carcinoma.


perivascular epithelioid cell neoplasm, angiomyolipoma, liver transplantation, hepatocellular carcinoma, mTOR inhibitors

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