Primary Perivascular Epithelioid Cell Tumor (PEComa) of the Liver: A Case Report and Review of the Literature

ABSTRACT

Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor consisting of distinctive perivascular epithelioid cells, and is commonly detected in the uterus. The liver is an uncommon site for primary PEComa. In this study, we report a case of primary hepatic PEComa in a 36-year-old woman. Upon gross examination, the tumor was a well-defined, brownish solid mass, measuring 6.5x5.2x4.5 cm. Microscopically, the tumor consisted largely of epithelioid cells and some spindle cells with a clear to eosinophilic cytoplasm and a rich network of delicate capillaries in the stroma. With the exception of their relatively large size and microscopically sinusoidal infiltrative growth pattern, all other histopathologic features of the tumor were consistent with their being benign. The tumor cells were positive for human melanoma black-45 and smooth muscle actin, and negative for cytokeratin-cocktail and c-kit.