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DX: Multilocular cystic renal cell carcinoma
1.The cysts are usually lined by a single layer of epithelial cells or lack an epithelial lining. The lining cells may be flat or plump and their cytoplasm ranges from clear to pale. Occasionally, the lining consists of several layers of cells or a few small papillae are present .The nuclei almost always are small, spherical,and have dense chromatin.
2. The septa consist of fibrous tissue, often densely collagenous. Within some of the septa there is a population of epithelial cells with clear cytoplasm. The epithelialcells resemble those lining the cysts and almost always have small dark nuclei.The clear cells form small collections but do not form expansile nodules. These epithelial cells often closely resemble histiocytes, or lymphocytes surrounded by retraction artefacts. Increased vascularity within the cell clusters is a clue to their nature.
此例最终诊断为:(左侧)多房性囊性肾细胞癌(MCRCC),也叫多囊性透明细胞肾细胞癌(RCC)和多囊性肾细胞癌;
免疫组化结果是:AE1/AE3(3+),CD68(-),Vimentin(-),CD10(+),RCC(+),Ki-67(+2%)
(1)MCRCC是一种预后极佳的少见的肾脏囊性肿瘤;2004年WHO肾肿瘤分类中将MCRCC作为透明细胞肾细胞癌的一种罕见亚型,其预后极佳。
(2)肿瘤直径1-14cm,平均4.9cm,68%的肿瘤Fuhrman分级多为Ⅰ级;5年生存率约100%;
(3)MCRCC应与多囊肾、节段性囊性疾病、囊性肾瘤和其他囊性肾细胞癌鉴别;
(4)近年来有作者建议应将MCRCC从新命名和分类,以提示该肿瘤大多数为良性。推荐使用低度恶性潜能的多囊性肾细胞癌作为这个病变的合适名称。