CAJ | 학술논문

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.
목적 제고신상피양혈관평활기지방류(EAML)적진치수평.방법 신EAML환자5례.남3례,녀2례.평균년령46(26～64)세.환측요부창통3례,체검우연발현2례.종류직경2.9～10.1 cm.B초화CT검사진단위혈관평활기지방류2례,신암、신상선종류、간암각1례.행신종류완제술3례,신설형절제술1례,신절제술1례.결과 병리보고:종류유불동비례적이상후벽혈관、평활기화지방조직구성.상피양세포포질농밀기산성혹투량,유기산성과립,병유봉부적혈관,상피양세포족상위요혈관.조직학형태표현위원형、다변형화사형상피양세포조성분포우지방류중,상피양류세포면역조화표기SMA、Vimentin、HMB45표체양성.5례병리진단균위신EAML.수방2～12개월,미견국부복발급원처전이.결과 신EAML유후벽혈관、지방화평활기세포혼합조성,상피양세포족상위요혈관.학진의뢰우림상、영상학급병이학검사상결합,면역조직화학염색유조우진단급감별진단.조기진단급완정절제종류시치료관건.
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.