Perret L, Chaubert P, Hessler D, Guillou L
University Institute of Pathology, Lausanne, Switzerland.
Cancer. 1998 Apr 15;82(8):1535-49.
Heterologous carcinosarcomas of the urinary bladder are rare neoplasms, the histogenesis of which is still disputed.
The clinicopathologic, immunohistochemical, and ultrastructural features of eight cases were analyzed.
The patients, 5 males and 3 females, had a median age of 70 years. Gross hematuria, dysuria, and urinary tract infections were the main presenting symptoms. Tumors were solitary in all cases and located in the right wall in six cases. Tumor size ranged from 2 to 12 cm (median, 5 cm). Four patients died of disease 2, 6, 17, and 42 months postoperatively, respectively. Microscopically, the tumors consisted mostly of a varied mixture of high grade transitional cell carcinoma with chondrosarcoma, osteosarcoma, rhabdomyosarcoma, and undifferentiated spindle cell (leiomyosarcoma-like) components with occasional transitional features between one component and another. All tumors but one invaded the muscularis propria or the perivesical fatty tissue. Immunohistochemically, keratin expression was observed focally in the sarcoma component as well as the carcinoma component. Reactivity for vimentin, desmin, muscle specific actin, and S-100 protein was observed in poorly differentiated areas in addition to the expected positivity of each histologic subtype of sarcoma. Ultrastructurally, one tumor showed evidence of both epithelial and chondrosarcomatous differentiation, whereas three contained rhabdomyosarcomatous elements.
On the basis of the current series and a review of 55 reports from the literature, primary heterologous carcinosarcoma of the urinary bladder proved to be a highly malignant type of neoplasm occurring predominantly in elderly males that was most often in an advanced stage at presentation and rapidly became lethal. Histogenetically, some heterologous carcinosarcomas should be regarded as a variant of sarcomatoid carcinoma (metaplastic carcinoma) that shows prominent heterologous differentiation.
膀胱异源性癌肉瘤是一种罕见的肿瘤，其组织发生仍存在争议。
分析8例患者的临床病理、免疫组化及超微结构特征。
患者共8例，男性5例，女性3例，中位年龄70岁。主要临床表现为肉眼血尿、排尿困难和尿路感染。所有病例肿瘤均为单发，6例位于右壁。肿瘤大小为2至12厘米（中位值5厘米）。4例患者分别于术后2、6、17和42个月死于本病。显微镜下，肿瘤主要由高级别移行细胞癌与软骨肉瘤、骨肉瘤、横纹肌肉瘤和未分化梭形细胞（平滑肌肉瘤样）成分混合而成，各成分之间偶尔可见移行特征。除1例肿瘤外，其余均侵犯固有肌层或膀胱周围脂肪组织。免疫组化显示，角蛋白在肉瘤成分和癌成分中均有局灶性表达。波形蛋白、结蛋白、肌肉特异性肌动蛋白和S-100蛋白除在肉瘤各组织学亚型预期阳性区域表达外，在低分化区域也有表达。超微结构上，1例肿瘤显示上皮和软骨肉瘤分化证据，3例含有横纹肌肉瘤成分。
基于本系列研究及对文献中55篇报道的回顾，膀胱原发性异源性癌肉瘤是一种高度恶性肿瘤，主要发生于老年男性，就诊时多处于晚期，且迅速致死。组织发生学上，部分异源性癌肉瘤应被视为肉瘤样癌（化生癌）的一种变体，具有显著的异源性分化。
