Abstract
(P)erivascular (E)pithelioid (C)ells-omas are mesenchymal tumors composed of peri-vascular epithelioid cells (PECs), a distinct group of cells which are often associated with the blood vessel wall and which have the peculiar characteristic of simultaneously ex-pressing melanocytic and smooth muscle immunohistochemical markers [1]. Actually, entity as “angiomyolipoma”, “epithelioid angiomyolipoma” and “lymphangioleiomy-omatosis” are included in this group, but, from 2020, with the release of the new classifi-cation of the World Health Organization, the use of old definitions such as "clear cell my-omelanocytic tumor" or "sugar tumor of the lung" is not raccomended [1]. PEComas are rare and more frequent in females than males with M:F ratio: 0.2:1, with a wide age range and a peak in young to middle-aged adults (mean age: 45 years) [2-5]. Primary cutaneous PEComa (cPEComa) are rare, described firstly by Crowson et al. [6] and occur as a painless lesion predominantly in female patients with a wide age range [7]. The majority of cPEComa arise on the extremities (lower>upper) and, less frequently, on the trunk [7-8]. In a very recent review [9], 65 cases of cPEComa were isolated from an in-depth analysis. In this paper we present a new case of cPEComa found on the left thigh of a 53-year-old pa-tient, we report the histopathological, immunohistochemical and molecular biology char-acteristics, and, finally, we compare our case with the cases previously reported in the literature.
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