多房性囊性肾细胞癌6例及临床病理分析

目的探讨多房性囊性肾细胞癌（MCRCC）的临床病理特征及鉴别诊断。方法对6例MCRCC进行病理形态及免疫组化观察，总结其临床资料，并进行文献复习。结果6例患者中男性4例，女性2例，年龄31—55岁，平均年龄45．5岁，术后随访6-49月，均无复发或转移。肿瘤均有包膜，完全由大小不等、互不相通的囊腔构成。裳腔内衬透明细胞，囊壁纤维间隔内有透明细胞巢，肿瘤性透明细胞核Fuhrman分级为Ⅰ级，免疫组化示AE1／AE3（＋）、EMA（＋）、CD15（-）、CD68（-）。结论多房性囊性肾细胞癌有独特的病理改变和良好的顸后。术前诊断中常难与其它囊性肾疾病鉴别，术后病理检查的关键特点是肉眼无膨胀性实性结节及镜下间隔内可见聚集的透明细胞巢。

Clinical analysis of multilocular cystic nephroma

Objective To investigate the clinicopathological features and differential diagnosis of multilocular cystic renal cell carcinoma （MCRCC）. Methods The pathomorphological change and immnnohistochemical stains of 6 cases of MCRCC were observed and their clinical data were summarized with the review of relevant literature. Results There were 6 MCRCC cases including 4 males and 2 females. The ages of these patients ranged from 31 to 55 years old and the mean age was 45.5 years old. After a followed up of 6 - 49 months no evidence of recurrence and metastasis was discovered. The MCRCC was surrounded by fibrous capsule and characterized by multiple non - communicating cysts with various size, the cysts were lined with clear cells, and in the septa there were some clear cells that arranged in cellular nest. All 6 MCRCC cases showed Fuhrman grade 1 nuclear features. Immunohistochemically, the clear cells were positive for AE1/AE3 and EMA, but negative for CD15 and CD68. Conclusion MCRCC has distinctive pathological change and a good prognosis. The preoperative diagnosis of MCRCC is often difficult to be differentiated from other cystic renal diseases. The critical features of the pathological change are the absence of any grossly visible expansive solid nodule of carcinoma cells and microscopically the neoplastic clear cells within the septa.