摘要目的 探讨肾脏黏液性管状和梭形细胞癌的临床病理学特点.方法 分析7例肾脏黏液性管状和梭形细胞癌的临床特点、组织形态及免疫表型特点[CD10、CK7、CK18、CK19、Villin、上皮细胞膜抗原(EMA)、P504S和波形蛋白],并复习相关文献.结果 7例黏液性管状和梭形细胞癌中,男性3例,女性4例,平均年龄48.2岁(39～61岁).均为体检时发现肿瘤,肿瘤最大径平均5.5 cm(4.0～9.0 cm),术后随访18～51个月,得到随访资料的5例均无复发及转移.肿瘤大体切面均为实性、灰白色,无包膜,但与周围肾组织分界清晰.镜下观察肿瘤细胞主要由两种形态构成:均一的由立方细胞构成的紧密排列的小管状结构和梭形细胞成分.两种成分比例或多或少,交错分布,其中5例伴有黏液样基质,3例见到明显的透明细胞区域,1例可见灶状肉瘤样区域,1例见乳头状结构及泡沫细胞.免疫组织化学染色显示,7例肿瘤CK7均呈阳性表达,EMA、CK18和P504S在染色的5例中全部呈阳性表达,CK19在染色的5例中有4例表达,而CD10、Villin和波形蛋白表达差异较大.结论 肾脏黏液性管状和梭形细胞癌是一种低度恶性的多形性肿瘤,组织形态学谱系较宽,不典型的病例可以主要由两种成分之一构成,并缺少黏液,有些病例可见到透明细胞、乳头状结构,少数可见肉瘤样形态及坏死.免疫表型上对于从近曲小管到集合管的标志物均有表达.

abstractsObjective To investigate the clinical and pathological features of the mucinous tubular and spindle cell carcinoma (MTSCC) of the kidney. Methods Seven cases of MTSCC were analyzed by gross examination and light microscopy. Immunostaining was performed to detect the expression of CD10, CK7, CK18, CK19, Villin, EMA, P504S and vimentin. The literature on this tumor was reviewed to discuss the histological features of MTSCC and its clinical behavior. Results Three of 7 cases were male and the other 4 were female. The mean age of the patients was 48.2 years old, with a range from 39 to 61 years. All the patients presented no symptom and their tumors were found by health examination. Tumors averaged 5.5 cm in greatest dimension (range from 4.0 cm to 9.0 cm). The tumors were well-circumscribed without capsules, and the cut surfaces were solid and soft with white-tan color. By light microscopy, tumors were composed of tightly packed, small, elongated tubules with transitions to spindle cell components. Five cases had mucinous stroma. Clear cell clusters, focal sarcomatoid differentiation, papillations and foamy macrophages were seen in several cases. Immunohistochemically, all 7 cases showed positive for CK7, five of 5 cases positive for EMA, CK18 and P504S, four of 5 cases positive for CK19, but heterogeneous for CD10, villin and vimentin expression. No evidence of local recurrence or distant metastases was identified in the 5 patients with follow-up information. Conclusions The mucinous tubular and spindle cell carcinoma is a low-grade and polymorphic neoplasm. The morphology of these tumors may not be uniform with a wide histological spectrum. The tumors can be tubular predominant or spindle cells predominant with scant to abundant mucinous stroma, which coupled with the presence of other unusual features such as clear cells, papillations, foamy macrophages, necrosis and sarcomatoid differentiation. Immunohistochemically, MTSCC can express the markers from the proximal convoluted tubules to collecting tubules.