PEComas localized in the region of falciform ligament and broad ligament are exceedingly rare. Most of them are built of spindle neoplastic cells. We report a case of epithelioid PEComa of the falciform ligament and/or broad ligament. There is only one report of such neoplasm in English-language literature. Histologically, the tumor was composed of nests of epithelioid clear cells stained positively for vimentin, HMB45, and SMA. Because of morphological features of the tumour (4 mitoses /20HPF, focal necrosis, and vascular invasion) we assess the neoplasm as potentially malignant.
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