Danilewicz Marian, Strzelczyk Janusz M, Wagrowska-Danilewicz Małgorzata
Pol J Pathol. 2017;68(1):92-95. doi: 10.5114/pjp.2017.67623.
Perivascular epithelioid cell tumor (PEComa) is a very rare lesion and is described by the World Health Organization (WHO) as a mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. In this report we describe PEComa with perirenal manifestation, which is exceedingly rare and to our best knowledge up to now worldwide only three cases have been described. Despite the reports that most PEComas are benign, this tumor met criteria for malignancy and coexisted with mucinous gallbladder cancer and nonresectable pancreatic head tumor. We concluded that despite the rarity of perirenal PEComas, in cases with an unusual epithelioid histological pattern the diagnosis of PEComa should also be taken into consideration on the basis of the immunohistochemical study.
血管周上皮样细胞瘤（PEComa）是一种非常罕见的病变，世界卫生组织（WHO）将其描述为一种间叶性肿瘤，由组织学和免疫组化上独特的血管周上皮样细胞组成。在本报告中，我们描述了具有肾周表现的PEComa，这极其罕见，据我们所知，迄今为止全世界仅报道过3例。尽管有报道称大多数PEComa是良性的，但该肿瘤符合恶性标准，并与黏液性胆囊癌和不可切除的胰头肿瘤并存。我们得出结论，尽管肾周PEComa罕见，但在具有不寻常上皮样组织学模式的病例中，也应根据免疫组化研究考虑PEComa的诊断。
