泌尿系统肉瘤样癌的临床病理特点与预后分析

Clinicopathologic features and prognosis analysis of sarcomatoid carcinoma of the urinary system

目的探讨泌尿系统肉瘤样癌的临床病理特点与预后。方法回顾性分析我院泌尿外科收治的10例泌尿系统肉瘤样癌患者的临床资料,均经影像学检查诊断为泌尿系肿瘤。7例行肾根治性切除术,1例行肾根治性切除术加下腔静脉切开取栓术,1例行肾、输尿管及部分膀胱切除术,1例行根治性全膀胱切除术。10例均经病理学确诊。术后4例行化疗,1例行放疗加化疗,1例行放疗加白细胞介素(白介素)治疗,4例未行放疗和化疗。观察患者预后。结果大体观肾肿瘤直径3.5~10.5 cm,质地脆,切面呈暗红或灰黄色,可见出血坏死。与肾实质分界不清,肿块部分与肾被膜粘连。膀胱肿瘤直径约5.5 cm,切面灰白质中,肉眼侵及肌层,周围膀胱黏膜灰黄,略粗糙。镜下观由大量梭形细胞组成,瘤细胞异型性明显,呈上皮样或多角形,排列呈束状,胞质丰富、红染或空淡透明。胞核圆形或卵圆形,深染,核分裂象易见。细胞角蛋白(CK)(+)、上皮细胞膜抗原(EMA)(+)、波形蛋白(Vimentin)(+)、S100(-)、平滑肌肌动蛋白(SMA)(-)、结蛋白(desmin)(-)。病理学诊断肾实质肉瘤样癌8例(单纯性肉瘤样癌7例、透明细胞癌伴肉瘤样癌1例),肾盂肉瘤样癌1例,膀胱尿路上皮癌伴肉瘤样癌1例。影像学诊断8例诊断为肾肿瘤,1例诊断为肾盂肿瘤,1例诊断为膀胱肿瘤。本研究9例患者死亡,其中8例生存周期为3~8个月;1例(透明细胞癌伴肉瘤样癌)生存周期为38个月,1例仍在随访中(膀胱尿路上皮癌伴肉瘤样癌),已生存50个月。结论泌尿系统肉瘤样癌罕见,发病年龄较大,恶性程度高,易侵犯局部脏器及血管,且易发生远处转移,对放化疗不敏感,预后差。

Objective To discuss the clinicopathologic features and prognosis of sarcomatoid carcinoma of the urinary system.Methods The clinical data of 10 patients with sarcomatoid carcinoma of the urinary tract admitted to urology department were retrospectively analyzed,all of whom were diagnosed as tumors of the urinary system by imaging.7 cases underwent radical nephrectomy,1 case underwent radical nephrectomy plus inferior vena cava thrombectomy,1 case underwent nephrectomy,ureterectomy and partial cystectomy,and 1 case underwent radical cystectomy.All 10 cases were confirmed by pathology.After surgery,4 cases received chemotherapy,1 case received radiotherapy plus chemotherapy,1 case received radiotherapy plus interleukin therapy,and 4 cases received no radiotherapy or chemotherapy.The prognosis of patients was observed.Results The renal tumor was 3.5-10.5 cm in diameter,with a brittle texture,dark red or grayish yellow cut surface,and visible hemorrhagic necrosis.The bladder tumor was approximately 5.5 cm in diameter,the invasion of the muscle layer was visible to the naked eye in the section of cinereum matter,and the surrounding bladder mucosa was grayish-yellow and slightly rough.Microscopically,they consist of a large number of spindle-shaped cells with obvious heterogeneity,epithelioid or polygonal shape,arranged in bundles,and abundant cytoplasm,redstained or empty pale transparent.The nuclei were round or ovoid,deeply stained,and nuclear fission was easily seen.Cytokeratin(CK)(+),epithelial membrane antigen(EMA)(+),Vimentin(+),S100(-),smooth muscle actin(SMA)(-),and desmin(-).Pathology diagnosed 8 cases of sarcomatoid carcinoma of the renal parenchyma(7 cases of simple sarcomatoid carcinoma and 1 case of clear cell carcinoma with sarcomatoid carcinoma),1 case of sarcomatoid carcinoma of the renal pelvis,and 1 case of urothelial carcinoma of the bladder with sarcomatoid carcinoma.The imaging diagnosis was renal tumor in 8 cases,renal pelvis tumor in 1 case,and bladder tumor in 1 case.9 patients died in this study,of which 8 cases had a survival cycle of 3-8 months,1 case(clear cell carcinoma with sarcomatoid carcinoma)had a survival period of 38 months.1 case was still under follow-up(urothelial carcinoma of the bladder with sarcomatoid carcinoma)and had survived for 50 months.Conclusion Sarcomatoid carcinoma of urinary system is rare,with older age of onset,high malignancy,easy to invade local organs and blood vessels,and prone to distant metastasis,insensitive to radiotherapy and chemotherapy,and has a poor prognosis.