肾嫌色细胞癌42例临床病理观察及免疫表型分析

Chromophobe renal cell carcinoma： a clinicopathologic study and immunophenotypes of 42 cases

目的 探讨肾嫌色细胞癌的临床病理学特征、免疫表型特点及预后.方法 收集2003至2011年青岛大学医学院附属医院、解放军第四○一医院及青岛市立医院共42例嫌色细胞癌的临床资料,分析其临床病理特点,进行Hale胶体铁染色,并采用免疫组织化学（EnVision法）检测相关免疫标志物.同时与40例肾透明细胞癌及10例嗜酸细胞瘤作对照.结果 男性17例,女性25例,年龄39 ～ 78岁（平均57岁）.肿瘤大小为2～ 19 cm（平均7.3 cm）.镜下观察：瘤组织主要呈实性片状排列,由胞质空亮、细网状排列的嫌色细胞或胞质嗜酸、具核周空晕的嗜酸细胞构成.瘤细胞大小不一,核不规则,核仁不明显,核分裂象较少见.所有肿瘤对Hale胶体铁染色均为阳性.免疫表型：嫌色细胞癌高表达上皮细胞膜抗原（ EMA,100.0％,42/42）、CK7（ 95.2％,40/42）、肾脏特异性钙黏素（ Ksp-cad,92.9％,39/42）、CK18（88.1％,37/42）和CD117（61.9％,26/42）,CD10和PAX2阳性率为31.0％ （13/42）和28.6％（12/42）.波形蛋白较少表达,而CAⅨ和TFE3均不表达.31例得到随访,随访时间2 ～ 77个月（平均29个月）,3例分别于术后3、8及13个月死于肿瘤转移,28例健在.结论 肾嫌色细胞癌好发于中老年人,预后较好.植物细胞样排列、大小不等、多少不一的嫌色细胞和具核周空晕的嗜酸细胞是本瘤重要的形态特征.Hale胶体铁染色及免疫组织化学染色EMA、CK7、Ksp-cad和CD117阳性对肾嫌色细胞癌的病理诊断及鉴别诊断具有重要作用.

Objective To study the clinicopathologic features,immunohistochemical profiles and prognosis of chromophobe renal cell carcinoma （ChRCC）. Methods Forty-two cases of ChRCC were retrieved from the archival files of the Affiliated Hospital of Qingdao University,401 Hospital of PLA and Qingdao Municipal Hospital from 2003 to 2011. The clinical and pathologic features of the tumors were reviewed.Hale colloidal iron staining was performed and EnVision immunohistochemistry was used to detect the expression of a series of immunologic markers.Forty cases of clear cell renal cell carcinoma and 10 cases of renal oncocytoma were selected as controls.Results The patients included 17 males and 25 females.The age of patients ranged from 39 years to 78 years （median age =57 years）.On gross examination,the tunors ranged from 2 cm to 19 cm in greatest dimension （mean size =7.3 cm）.Histologically,the tumors were mainly composed of solid sheets,acini or tubules of malignant cells.The tumor cells contained clear finely reticular （ “chromophobe” ） and eosinophilic cytoplasm with perinuclear clearing.The nuclear outline was irregular and wrinkled.Nucleoli were inconspicuous and mitotic figures were barely seen.Hale colloidal iron stain was positive in all cases,Immunohistochemically,the tumor cells were variably positive for EMA （ 100%,42/42）,CK7 （ 95.2%,40/42 ）,Ksp-cad （ 92.9%,39/42 ）,CK18 （ 88.1%,37/42 ）,CD117（61.9,26/42）,CD10 （31.0%,13/42 ） and PAX2 （ 28.6%,12/42 ）.They were negative for vimentin,CA Ⅸ and TFE3.The follow-up period in 31 patients ranged from 2 to 77 months （ average duration =29months）.Three patients died of tumor metastasis 3,8,13 months respectively after the operation.Twentyeight patients were still alive without evidence of tumor recurrence.Conclusions ChRCC predominantly occurs in middle-aged and elderly patients.It often carries a favorable prognosis.The presence of plant celllike morphology,pale cells with uniform reticular microvesicular appearance and perinuclear clearing are characteristic histologic features. The diffuse positivity for Hale colloidal iron stain and EMA/CK7/Kspcadherin/CD117-positive immunoprofiles are also useful in differential diagnosis.