Shen Steven S, Ro Jae Y, Tamboli Pheroze, Truong Luan D, Zhai Qihui, Jung Soo-Jin, Tibbs Rita G, Ordonez Nelson G, Ayala Alberto G
Department of Pathology, The Methodist Hospital and Research Institute, Houston, TX 77030, USA.
Ann Diagn Pathol. 2007 Feb;11(1):13-21. doi: 10.1016/j.anndiagpath.2006.09.005.
Mucinous tubular and spindle cell carcinoma is a rare and newly described type of renal cell carcinoma (RCC) with a relatively indolent behavior. However, its histogenetic origin or line of differentiation remains unclear. Twelve cases of mucinous tubular and spindle cell carcinoma were identified and retrieved from the files of 3 institutions. Detailed morphological features, as well as their immunohistochemical profile established with markers of proximal renal tubules (RCC marker antigen, CD15, and alpha-methylacyl-CoA racemase) and of distal renal tubules (kidney-specific cadherin and cytokeratin 7), were studied. The age range of the patients was 35 to 73 years with a median of 56 years. The male to female ratio was 1:3. All of the patients were alive with follow-up between 4 and 38 months. All the tumors were confined to the kidney with a mean tumor size of 6.9 cm (range, 1.8-17 cm). The tumors were composed of variable proportions of tubular and spindle cell areas with focal to prominent mucinous or myxoid stroma. Foamy macrophages were seen in 10 cases and were prominent in 4 cases. A focal compressed tubulopapillary growth pattern was seen in 10 cases. The tumor cells were uniformly cuboidal with ovoid to round nuclei and inconspicuous nucleoli (Furhman nuclear grade 3 in 6 cases). Focal necrosis was seen in 3 cases. Immunostains showed that tumors were positive for RCC marker antigen (11/12), alpha-methylacyl-CoA racemase (11/12), CD15 (8/12), CD10 (2/12), kidney-specific cadherin (1/12), and cytokeratin 7 (11/12). Its morphological features as well as a strong preferential expression of proximal tubule markers suggest that this tumor is a type of RCC with proximal tubular differentiation, which appears closely related to or represents a morphological variant of papillary RCC.
黏液性小管状和梭形细胞癌是一种罕见的、新描述的肾细胞癌（RCC）类型，其行为相对惰性。然而，其组织发生起源或分化谱系仍不清楚。从3家机构的档案中识别并检索出12例黏液性小管状和梭形细胞癌。研究了详细的形态学特征，以及用近端肾小管标志物（RCC标志物抗原、CD15和α-甲基酰基辅酶A消旋酶）和远端肾小管标志物（肾特异性钙黏蛋白和细胞角蛋白7）建立的免疫组化谱。患者年龄范围为35至73岁，中位年龄为56岁。男女比例为1:3。所有患者均存活，随访时间为4至38个月。所有肿瘤均局限于肾脏，平均肿瘤大小为6.9 cm（范围为1.8 - 17 cm）。肿瘤由不同比例的小管状和梭形细胞区域组成，伴有局灶性至显著的黏液性或黏液样间质。10例可见泡沫状巨噬细胞，4例显著。10例可见局灶性压缩性小管乳头状生长模式。肿瘤细胞均为立方形，核呈卵圆形至圆形，核仁不明显（6例为福尔曼核分级3级）。3例可见局灶性坏死。免疫组化显示肿瘤RCC标志物抗原阳性（11/12）、α-甲基酰基辅酶A消旋酶阳性（11/12）、CD15阳性（8/12）、CD10阳性（2/12）、肾特异性钙黏蛋白阳性（1/12）、细胞角蛋白7阳性（11/12）。其形态学特征以及近端小管标志物的强烈优先表达表明，这种肿瘤是一种具有近端小管分化的RCC类型，似乎与乳头状RCC密切相关或代表其形态学变异型。
