Liver transplantation for hepatic PEComa mimicking hepatocellular carcinoma

Česká verze

Authors: Selucká J. ¹; Jiří Froněk ² ; L. Voska ³ ; D. Kautznerová ⁴ ; P. Taimr ¹
Authors place of work: Klinika hepatogastroenterologie, IKEM, Praha 2 Klinika transplantační chirurgie, IKEM, Praha 3 Pracoviště klinické a transplantační patologie, IKEM, Praha 4 Pracoviště radiodiagnostiky a intervenční radiologie, IKEM, Praha ¹
Published in the journal: Gastroent Hepatol 2022; 76(2): 121-126
Category: Kazuistika
doi: https://doi.org/10.48095/ccgh2022121

Summary

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.

Keywords:

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

Zdroje

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