13例多房性囊性肾细胞癌的临床病理特点分析

Analysis on Features of Clinical Pathology in 13 Cases with Multilocular Cystic Renal Cell Car cinoma

目的探讨多房性囊性肾细胞癌(MCRCC)的临床病理特点。方法收集13例MCRCC的资料,进行临床特点,形态学和免疫组织化学比较。结果 13例患者中男性9例,女性4例,男女比例2.25:1。患病年龄26-68岁,平均年龄47.2岁。临床上多为偶然或体检发现,偶有腰痛症状。形态学检查:肿瘤最大径1.7-6cm,囊性或局灶实性,境界清楚,切面均呈多房性或蜂窝状,部分囊内含灰黄色或血性液体。镜下观察:囊腔大多衬覆单层肿瘤细胞,部分为多层,胞质透明、核小、未见明显核仁、Fuhrman核分级Ⅰ级,局灶囊内有小乳头结构。大多数病例中纤维间隔内可见与囊腔衬覆细胞类似的透明细胞团,多呈巢状。免疫组织化学染色:肿瘤细胞对免疫组化标记AE1/3,EMA,Vimetin均呈弥漫阳性表达,8例的CK7及10例的CD10肿瘤细胞呈阳性或局灶阳性,P504S有3例阳性或局灶阳性表达,CK20,CD117,CD163均呈阴性,Ki-67指数达1%-5%。P53中有11例阴性表达,2例有1%肿瘤细胞阳性表达。结论 MCR CC是一种少见的肾细胞癌组织学亚型,预后良好,免疫组化标记AE1/3,EMA,Vimetin,CK7,CK20,CD10等对其诊断及鉴别诊断有帮助。

Objective To investigate the clinicopathological features of multilocular cystic renal cell carcinoma（MCRCC）, and to improve the clincopathological features of this disease. Methods 13 cases MCRCC data, clinical characteristics, pathological features and also immuniophenotype were collected. Results In 13 patients： 9 of them were males and 4 were females, the ratio is 2.25： 1. The prevalence o age was at a range of 26- 68 years, mean age is 47.2 years. Mostly of them were found by incidental or only back pain symptoms occasionally. Morphological examination： maximum of tumors is at a range of 1.7- 6cm. They were cystic or focal cystic solid,have a clear boundary. They showed as multilocular or honeycomb, part of them containing yellow or bloody fluid. Microscopic observation：Most cysts lined by a single layer of tumor cells, multi- part perhaps, with clear cytoplasm, and the nuclear small, have no obvious nucleoli.According to Fuhrman nuclear grade,they were level I; also,we can see focal cystic with small papillary structures. Most cases can be seen and cysts lined by cells similar to clear cell groups, mostly nested within the fibrous septa. Immunohistochemical staining： tumor cells markered with AE1 / 3, EMA, Vimetin showed diffuse expression, CD10 cells were eight cases of tumor and 10 cases of CK7 positive or focally positive, P504 S has three were positive or focally positive expression, CK20, CD117, CD163 were negative, Ki- 67 index of 1%- 5%. P53 negative expression in 11 cases, two cases have positive 1% of tumor cells. Conclusion MCRCC is a rare histologic subtypes of renal cell carcinoma, the prognosis is good, immunohistochemical markers AE1 / 3, EMA, Vimetin, CK7, CK20, CD10 and other help for its diagnosis and differential diagnosis.