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目的 探讨尿路上皮肉瘤样癌的临床及病理学特征. 方法 回顾性分析1999年1月至2010年12月经病理诊断的尿路系统肉瘤样癌18例患者的临床资料,男11例,女7例.年龄41～84岁,平均63岁.均有无痛性肉眼血尿,伴腰部不适3例,伴尿路刺激症状4例,伴尿路梗阻5例.13例发生于膀胱,4例发生于输尿管,1例为输尿管和肾盂多发肿瘤.其中肾盂、输尿管多发肿瘤患者于2002年曾行同种异体肾移植手术,术后一直服用抗排斥药物. 结果 本组18例行全膀胱根治切除术9例,行肾及输尿管切除术5例,行膀胱肿瘤电切术2例,行膀胱肿瘤激光汽化切除术1例,行膀胱肿瘤活检术1例.肿瘤直径2.5～12.0 cm,平均5.6 cm.镜下均可见高级别尿路上皮癌成分,其中伴肉瘤样分化12例,伴鳞状细胞癌及肉瘤样分化3例,伴腺癌及肉瘤样分化3例,肉瘤样成分占肿瘤体积的10％～90％.免疫组化染色结果显示肉瘤样癌区域Vimentin(+),CK(+).本组死亡12例,术后生存时间20 d～14个月,中位生存期为7个月.3例术后分别随访3、3、17个月仍存活,失访3例. 结论 尿路系统肉瘤样癌恶性程度高,预后不良,确诊依赖病理及免疫组化检查.手术切除是首选治疗方式,配合化疗、放疗可提高疗效.

Objective To investigate the clinical and pathological characteristics of urocpithelial sarcomatoid carcinoma of the urinary system,and improve the diagnosis and treatment of these tumors.Methods We reviewed the pathology comfirmed urinary system caicinoma at the General Hospital of PLA from January 1999 to December 2010 and analyzed the clinical and pathological data of cases with sarcomatoid carcinoma in them retrospectively according to the literatures. Results A total of 4386 urinary system carcinoma were comfirmed,among them 18 (0.41％) cases of sarcomatoid carcinoma were identified,including 13 from bladder,4 from ureter and 1 multiple neoplasm of pelvis and ureter.The patients,11 males and 7 female,had a median age of 63.3 years (41-84 yrs).Gross hematuria (18 cases) were the main presenting symptoms,3 with flank pain,4 with pollakisuria,urgency and dysuria (urinary irritation symptoms] and 5 with urinary obstruction.The patient with multiple neoplasm of pelvis and ureter received allograft renal transplantation 7 years ago and took anti-rejection drugs all the way after operation.17 patients underwent surgical resection,9 radical cystectomy,5 radical nephroureterectomy,3 partial cystectomy and the remain was biopsy.Tumor size ranged from 2.5 to 12 cm (median,5.6 cm).Microscopically,coexisting high grade urothelial carcinoma was present in all the 18 cases,12 with sarcomatoid differentiation,3 with sarcomatoid and squamous carcinoma differentiation and 3 with sarcomatoid and glandular differentiation.Sarcomatoid component occupied 10 -90 percent.Immunohistochemical testing showed the sarcomatoid cells were positive for cytokeratin (CK) and vimentin.12 patients died of disease 20 days to 42 months (median,5.3 months) postoperatively.3 patients have been surviving with tumor free for 3,3 and 17 months respectinely,and the other 3 was failure to be followed. Conclusions Sarcomatoid carcinoma of the urinary bladder is a highly malignant disease,and the prognosis is very poor.The diagnosis depends on histopathologic and immunohistochemical studies.Early diagnosis and cystectomy or nephroureterectomy together with chemotherapy/radiotherapy may improve the curative effect.