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[具有横纹肌样特征的肾细胞癌的临床病理研究]

[Clinicopathologic study of renal cell carcinoma with rhabdoid features].

作者信息

Ma Jie, Zhou Xiao-jun, Huang Wen-bin, Zhou Hang-bo, Jiang Shao-jun, Rao Qiu, Lu Zhen-feng, Shi Qun-li

机构信息

Department of Pathology, Medical School of Nanjing University/Nanjing General Hospital of PLA, Nanjing 210002, China.

出版信息

Zhonghua Bing Li Xue Za Zhi. 2007 Mar;36(3):166-70.

Abstract

OBJECTIVE

To study the clinicopathologic features and biologic behavior of renal cell carcinoma (RCC) with rhabdoid features.

METHODS

Ten cases of RCC with rhabdoid features collected during the period from 1995 to 2005 were enrolled into the study. The clinical findings were analyzed and the hematoxylin and eosin-stained sections were reviewed. Immunohistochemistry and electron microscopy were also performed.

RESULTS

The age of patients ranged from 33 to 69 years (mean age = 52 years). Nine of the patients were males and 1 female. Five patients showed evidence of perinephric invasion. Two patients presented with regional lymph node metastases and 1 patient showed distant metastasis to the lung. Histologically, the rhabdoid foci were characterized by loosely cohesive trabeculae, acini, lobules and clusters of rhabdoid cells in otherwise clear cell RCC (9 cases) or papillary RCC (1 case). The rhabdoid cells were round to polygonal in shape and contained globular eosinophilic inclusion bodies in the cytoplasm, eccentric nuclei, vesicular chromatin pattern and prominent nucleoli. Coagulative tumor necrosis was commonly seen. Immunohistochemical study showed that the rhabdoid cells were diffusely positive for CD10 (10/10), cytokeratin AE1/AE3 (10/10), epithelial membrane antigen (10/10) and vimentin (10/10). Focal staining for neuron-specific enolase and S-100 protein was also noted. They were negative for CK7, CK20 and myogenic markers (including myogenin, smooth muscle actin and muscle-specific actin). The mean Ki-67 labeling index of the rhabdoid component was higher than that of the non-rhabdoid component (P < 0.05). Follow-up information was available in 8 patients. While 6 patients are still alive without recurrence, 2 patients died of the disease 6 and 29 months respectively after the operation.

CONCLUSIONS

RCC with rhabdoid elements are mainly observed in clear cell RCC and need to be distinguished from oncocytic renal tumors and malignant rhabdoid tumor of kidney. The higher proliferative activity in the rhabdoid areas may indicate more aggressive biologic behavior.

摘要

目的

研究具有横纹肌样特征的肾细胞癌(RCC)的临床病理特征及生物学行为。

方法

收集1995年至2005年期间的10例具有横纹肌样特征的RCC病例进行研究。分析临床资料并复查苏木精-伊红染色切片。同时进行免疫组织化学和电子显微镜检查。

结果

患者年龄在33至69岁之间(平均年龄=52岁)。9例为男性,1例为女性。5例有肾周侵犯证据。2例出现区域淋巴结转移,1例有肺远处转移。组织学上,横纹肌样病灶表现为在透明细胞RCC(9例)或乳头状RCC(1例)中,横纹肌样细胞松散聚集形成小梁、腺泡、小叶和细胞簇。横纹肌样细胞呈圆形至多边形,胞质内含有球形嗜酸性包涵体,核偏位,染色质呈泡状,核仁明显。常见凝固性肿瘤坏死。免疫组织化学研究显示,横纹肌样细胞CD10(10/10)、细胞角蛋白AE1/AE3(10/10)、上皮膜抗原(10/10)和波形蛋白(10/10)弥漫性阳性。还可见神经元特异性烯醇化酶和S-100蛋白局灶性染色。它们CK7、CK20和肌源性标志物(包括肌生成素、平滑肌肌动蛋白和肌肉特异性肌动蛋白)阴性。横纹肌样成分的平均Ki-67标记指数高于非横纹肌样成分(P<0.05)。8例患者有随访信息。6例患者仍存活无复发,2例患者分别在术后6个月和29个月死于该病。

结论

具有横纹肌样成分的RCC主要见于透明细胞RCC,需与嗜酸性肾肿瘤和肾恶性横纹肌样瘤相鉴别。横纹肌样区域较高的增殖活性可能提示更具侵袭性的生物学行为。

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