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目的探讨伴横纹肌样特征的肾细胞癌(RCC)的临床病理特征。方法对1995—2005年南京军区南京总医院常规外检档案352例 RCC 中的10例伴横纹肌样特征的 RCC 进行了光镜、免疫表型检测和超微结构观察,并结合临床病理特征进行分析。结果 10例伴横纹肌样特征的RCC 患者年龄33～69岁(平均年龄52岁),男9例,女1例。5例肿瘤侵犯肾包膜,2例伴淋巴结转移,1例伴肺转移。组织学观察,10例伴横纹肌样特征的 RCC 中透明细胞型9例,乳头型1例,均可见不同程度的具横纹肌样特征的瘤细胞区域。横纹肌样特征的瘤细胞排列成梁索状、腺泡样、器官样或团片状,瘤细胞散在分布,细胞间无黏附或黏附性差,呈类圆形或多角形,核泡状、偏位,核仁突出,胞质内见红染均质包涵体样物,瘤组织常伴明显坏死。免疫组织化学观察,具横纹肌样特征的瘤细胞CD10、CK(AE1/AE3)、上皮细胞膜抗原(EMA)、波形蛋白均阳性表达,CK7、CK20、结蛋白、肌细胞生成素、α-平滑肌肌动蛋白(α-SMA)、肌肉特异性肌动蛋白(MSA)均阴性表达,神经元特异性烯醇化酶(NSE)、S-100蛋白灶性阳性表达。横纹肌样瘤细胞区 Ki-67阳性表达较周围经典型瘤细胞区增高,两者比较差异有统计学意义(P<0.05)。随访8例,2例于术后6个月及29个月死亡,6例存活。结论伴横纹肌样特征的 RCC 主要见于透明细胞型,应与胞质嗜酸的肾细胞肿瘤和肾恶性横纹肌样瘤等相鉴别。RCC 中伴横纹肌样特征的瘤细胞较周围经典型瘤细胞 Ki-67表达明显增高,可能与此类肿瘤临床生物学行为更具侵袭性有关。
Objective To investigate the clinicopathological features of rhabdomyosarcoma with Rhabdomyosarcoma (RCC). Methods Ten cases of RCC with rhabdomyosarcoma in 352 cases of RCC routinely examined in Nanjing General Hospital of Nanjing Military Region from 1995 to 2005 were observed by light microscopy, immunophenotyping and ultrastructural observation combined with clinicopathological features . Results Ten patients with rhabdomyosarcoma of RCC were 33-69 years old (mean age 52 years), 9 males and 1 female. 5 cases of tumor invasion of renal capsule, 2 cases with lymph node metastasis, 1 case with lung metastasis. Histological observation showed that there were 9 cases of clear cell type and 1 case of papillary type in RCC of 10 patients with rhabdomyosarcoma. All of them showed tumor cells with rhabdomyosarcoma in different degree. Rhabdomyosarcoma cells arranged in a cord-like, acinar-like, organ-like or cluster-like, scattered tumor cells scattered between the cells without adhesion or poor adhesion was round or polygonal, bubble-like, offset , Prominent nucleoli, cytoplasm, see the red dye homogenous inclusion body samples, tumor tissue often accompanied by significant necrosis. The results of immunohistochemical staining showed that there were positive rhabdomyosarcoma CD10 and CK (AE1 / AE3), epithelial cell membrane antigen (EMA), vimentin, CK7, CK20, desmin, myogenin, The expressions of α-SMA, MSA, NSE and S-100 were all positive. The positive expression of Ki-67 in rhabdomyosarcoma cells was higher than that in the surrounding classic tumor cells, the difference was statistically significant (P <0.05). Eight patients were followed up. Two patients died at 6 months and 29 months after operation. Six patients survived. Conclusions RCC with rhabdomyosarcoma is mainly seen in clear cell type, which should be differentiated from cytoplasmic eosinophilic renal cell carcinoma and malignant rhabdomyosarcoma. Rhabdomyosarcoma-like features of the tumor cells than the surrounding typical tumor cells Ki-67 expression was significantly increased, and may be related to the clinical and biological behavior of these tumors more invasive.