Abstract
目的： 探讨肾混合性上皮和间质肿瘤（MEST）家族的临床病理学特征。 方法： 对MEST家族中2例成人囊性肾瘤和7例MEST的临床资料、组织病理学、免疫组织化学进行分析。 结果： 9例患者中，围绝经期妇女5例，男性3例和孕妇1例。可双肾多发。肿瘤大体为囊实性结构。镜下呈上皮和间质的双相性病变，上皮细胞为立方或扁平上皮、柱状上皮，胞质透明或嗜酸性，核圆形、杆状或多形性，单层或假复层排列成巢团状、腺样、乳头状、簇状等，腔内可见嗜酸性分泌物；间质由伴有平滑肌分化的梭形细胞组成，类似卵巢样间质。上皮细胞细胞角蛋白、PAX8阳性，CD10阴性；间质细胞雌激素受体、孕激素受体、波形蛋白、CD10、肌动蛋白、结蛋白、平滑肌肌动蛋白可为阳性，HMB45、Melan A为阴性。 结论： MEST家族是一种具有形态学差异、与性别相关肿瘤家族，病变中存在正常的肾小球，对肿瘤发病机制有重要提示价值。.
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