[1]张秀梅,罗亚岚,付明杰,等.肾恶性血管周上皮样细胞肿瘤临床病理特征及文献复习[J].医学信息,2020,33(03):168-170.[doi:10.3969/j.issn.1006-1959.2020.03.056]
　ZHANG Xiu-mei,LUO Ya-lan,FU Ming-jie,et al.Clinical and Pathological Characteristics of Malignant Renal Perivascular Epithelioid Cell Tumors and Literature Review[J].Medical Information,2020,33(03):168-170.[doi:10.3969/j.issn.1006-1959.2020.03.056]
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肾恶性血管周上皮样细胞肿瘤临床病理特征及文献复习()
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医学信息[ISSN:1006-1959/CN:61-1278/R]

卷:
33卷
期数:
2020年03期
页码:
168-170
栏目:
诊疗技术
出版日期:
2020-02-01

文章信息/Info

Title:
Clinical and Pathological Characteristics of Malignant Renal Perivascular Epithelioid Cell Tumors and Literature Review
文章编号:
1006-1959（2020）03-0168-03
作者:
张秀梅; 罗亚岚; 付明杰; 等
（1.大连医科大学附属大连市中心医院病理科，辽宁 大连 116033；2.大连医科大学附属第一医院腹部急症外科，辽宁 大连 116011）
Author(s):
ZHANG Xiu-mei1; LUO Ya-lan2; FU Ming-jie1; LI Ping1; ZHAO Jun-jun1
(1.Department of Pathology,Dalian Central Hospital,Dalian Medical University,Dalian 116033,Liaoning,China;2.Department of Abdominal Emergency Surgery,the First Affiliated Hospital of Dalian Medical University,Dalian 116011,Liaoning,China)
关键词:
肾; 恶性血管周上皮样细胞肿瘤; 鉴别诊断
Keywords:
Kidney; Malignant perivascular epithelioid cell tumor; Differential diagnosis
分类号:
R737.11
DOI:
10.3969/j.issn.1006-1959.2020.03.056
文献标志码:
A
摘要:
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。
Abstract:
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.

参考文献/References:

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更新日期/Last Update: 2020-02-01