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

Perivascular epithelioid cells (PECs) were initially proposed, by Bonetti et al. in 1992, to describe an “unusual atypical cell type” of epithelioid shape and harboring a clear to eosinophilic cytoplasm with perivascular distribution in both renal angiomyolipoma (AML) and clear cell sugar tumor (CCST) of the lung. This perception gradually became accepted and led to the concept of perivascular epithelioid cell tumors (PEComas). The PEComa family now embraces AML, CCST, lymphangiomyomatosis (LAM), and neoplasms composed predominantly of PECs have been designated by a variety of names, including clear cell myomelanocytic tumor (CCMMT), monotypic epithelioid AML, etc. Non-AML, non-LAM PEComa, a neoplasm composed solely of PECs, most commonly occurs in the uterus, but is relatively rarely detected in the liver. In this study, we describe a case of primary PEComa occurring in the liver.