肾上皮样血管平滑肌脂肪瘤诊治探讨

目的 提高肾上皮样血管平滑肌脂肪瘤(EAML)的诊治水平.方法 肾EAML患者5例.男3例,女2例.平均年龄46(26～64)岁.患侧腰部胀痛3例,体检偶然发现2例.肿瘤直径2.9～10.1 cm.B超和CT检查诊断为血管平滑肌脂肪瘤2例,肾癌、肾上腺肿瘤、肝癌各1例.行肾肿瘤剜除术3例,肾楔形切除术1例,肾切除术1例.结果 病理报告:肿瘤由不同比例的异常厚壁血管、平滑肌和脂肪组织构成.上皮样细胞胞质浓密嗜酸性或透亮,有嗜酸性颗粒,并有丰富的血管,上皮样细胞簇常围绕血管.组织学形态表现为圆形、多边形和梭形上皮样细胞灶性分布于脂肪瘤中,上皮样瘤细胞免疫组化标记SMA、Vimentin、HMB45表达阳性.5例病理诊断均为肾EAML.随访2～12个月,未见局部复发及远处转移.结果 肾EAML由厚壁血管、脂肪和平滑肌细胞混合组成,上皮样细胞簇常围绕血管.确诊依赖于临床、影像学及病理学检查相结合,免疫组织化学染色有助于诊断及鉴别诊断.早期诊断及完整切除肿瘤是治疗关键.

Epithelioid angiomyolipoma of the kidney

Objective To review the diagnosis and treatment of epithelioid angiomyolipoma of the kidney. Methods Five cases of epithelioid angiomyolipoma of the kidney were retrospectively analyzed in the aspects of imaging and pathologic characteristics. There were 3 males and 2 females with average age of 46 years (ranging from 26-64 years). There were 3 patients complained of pains of lumber. Two cases were found renal masses in routine physical examination. The mass diameter was 2.9-10. 1 cm. Two cases were diagnosed as angiomyolipoma and three as malignant tumors as kidney cancer, tumor of the adrenal gland or tumor of the liver with ultrasound and CT scan. Four of the 5cases accepted partial nephrectomy and 1 with radical nephrectomy. Results Epithelioid angiomyolipoma of the kidney was composed of abnormal thick parietal vessel, smooth muscle and adipose tissue in different proportional. The cytoplasm of epithelioid cells was acidophilia or lucency, which had acidophilia particles and abundant blood vessels. Epithelioid cells were round or polygon, surrounded to blood vessels, distributed in lipoid. The SMA, Vimentin and HMB45 were positive by immunohistochemisty. The post-operative histological diagnosis showed that all cases were epithelioid angiomyolipoma of the kidney. All patients were followed up for 2 to 12 months. No patients had locally recurrence or distant metastasis. Conclusions The diagnosis of epithelioid angiomyolipoma of the kidney relies on histopathology results. The immunohistochemical staining could be helpful for diagnosis and differential diagnosis.