Summary. Perivascular epithelioid cells tumors (PEComa) are rare neoplasm of mesenchymal origin that are histologically and immunohistochemically similar to perivascular epithelioid cells. PEComas do not have specific clinical picture, there are no specific criteria for malignancy. The definitive diagnosis is possible only after immunohistochemistry study. The case of uterus PEComa is presented.
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