肾原发性上皮样血管肉瘤伴肾盂移行细胞癌

作者报道应用临床病理学、免疫组织化学和电子显微镜检查，发现１例左肾原发性上皮样血管肉瘤伴肾盂移行细胞癌。该病例第八因子相关抗原、荆豆凝集素、内皮细胞抗体、波形蛋白、细胞角蛋白和上皮膜抗原（ＦⅧＲＡ、ＵＥＡ、ＣＤ３１、Ｖｉｍｅｎｔｉｎ、Ｃｙｔｏｋｅｒｅｔｉｎ和ＥＭＡ）阳性，电镜查见Ｗ－Ｐ小体。作者认为该病是一种罕见肿瘤，预后差，其形态学和免疫组织化学类似于上皮性肿瘤，易导致误诊。电镜和内皮性标志物ＦⅧＲＡ、ＣＤ３１、ＵＥＡ的联合应用可确诊本瘤。

Primary renal epithelioid angiosarcoma with transitional cell carcinoma in renal pelvis

To understand the clinical characteristics of primary renal epithelioid angiosarcoma. We used clinical pathology, immunohistochemistry and electron microscopy. Analysis and discussion about the case were combined with a review of the literature. We first report a female case of the left renal epithelioid angiosarcoma and concomitantly, transitional cell carcinoma developing from the renal pelvis at age 69. Epithelioid angiosarcoma showed positive results for FVIIIRA+, UEA, CD31, Cytokeratin and EMA. Weibel-Palade bodies were identified by electron microscopy. Primary renal epithelioid angiosarcoma was a rare clinical entity with a poor prognosis, which mimics epithelial tumor, both morphologically and immunohistochemically, and may be lead to misdiagnosis. The combined use of endothelial cell markers including FVIIIRA, CD31, UEA and electronmicroscopy can confirm the diagnosis of this neoplasm.