肾恶性血管周上皮样细胞肿瘤临床病理特征及文献复习

Clinical and Pathological Characteristics of Malignant Renal Perivascular Epithelioid Cell Tumors and Literature Review

目的分析3例肾恶性血管周上皮样细胞肿瘤(PEComa)的临床病理特征、免疫表型、诊断、鉴别诊断、治疗方式及预后。方法回顾性分析2010年1月~2018年12月我院诊断的3例肾恶性血管周上皮样细胞肿瘤的临床特征、组织学特点及免疫表型,并复习相关文献。结果3例肾恶性PEComa患者中,男性1例,女性2例,年龄42~50岁,平均年龄45.3岁。肿瘤最大直径4.3~12.5 cm,肿瘤细胞弥漫排列,以上皮样细胞为主,胞浆丰富,透亮或呈嗜酸性,细胞异型性明显,可见出血及大片坏死,核分裂像2~5个/50 HPF。免疫组化结果显示,3例肿瘤细胞HMB45均局灶阳性,Melan-A和SMA弥漫强阳性。结论肾恶性血管周上皮样细胞肿瘤是一种罕见的间叶源性肿瘤,预后较差,易复发和远处转移,主要依据组织学形态和免疫组化明确诊断。

Objective To analyze the clinicopathological characteristics,immunophenotype,diagnosis,differential diagnosis,treatment methods and prognosis of 3 cases of renal malignant perivascular epithelioid cell tumor(PEComa).Methods The clinical characteristics,histological characteristics,and immunophenotypes of 3 cases of renal malignant perivascular epithelioid cell tumors diagnosed in our hospital from January 2010 to December 2018 were retrospectively analyzed,and the relevant literature was reviewed.Results Among the 3 patients with renal malignant PEComa,1 was male and 2 were female,aged 42 to 50 years,with an average age of 45.3 years.The maximum diameter of the tumor is 4.3~12.5 cm.The tumor cells are diffusely arranged.The epithelial cells are dominant.The cytoplasm is rich,translucent or eosinophilic.The cell is atyptomorphic.Hemorrhage and massive necrosis can be seen.The mitotic image is 2~5/50 HPF.Immunohistochemical results showed that HMB45 was positive in all 3 tumor cells and Melan-A and SMA were diffusely positive.Conclusion Renal malignant perivascular epithelioid cell tumor is a rare mesenchymal tumor with poor prognosis,easy recurrence and distant metastasis.The diagnosis is mainly based on histological morphology and immunohistochemistry.