脐尿管癌的临床特点及疗效观察  

Clinical features and surgical outcomes of urachal carcinoma

作　　者：杨福江[1] 赵耀瑞[2] 王勇[1] 杨宪法[1] 郜海涛[1] 徐勇[1] 孙光[1] 

机构地区：[1]天津医科大学第二医院泌尿外科天津市泌尿外科研究所,300211 [2]武警后勤学院附属医院泌尿外科

出　　处：《现代泌尿生殖肿瘤杂志》2014年第5期268-272,共5页Journal of Contemporary Urologic and Reproductive Oncology

摘　　要：目的探讨脐尿管癌的临床特点及疗效,提高对该病的诊疗水平。方法回顾性分析2002年1月至2013年12月15例脐尿管癌患者的临床资料、治疗方案和随访结果。结果病理证实黏液腺癌7例,腺癌6例,黏液腺癌伴尿路上皮细胞癌1例,小细胞内分泌癌1例。依据Sheldon分期标准,Ⅱ期1例,ⅢA期9例,ⅢB期4例,ⅢC期1例。行扩大性膀胱部分切除术14例,其中同时行双侧盆腔淋巴结清扫1例,行膀胱部分切除术1例。12例获随访,失访3例,随访时间3-67个月,中位随访时间36个月。1例小细胞内分泌癌患者接受MEP方案化疗,于术后3个月死于全身骨转移;1例术后1年复发,行经尿道肿瘤电切术并接受联合化疗及盆腔放疗,于术后1.5年死于远处转移;1例术后15个月和2年复发,行经尿道肿瘤电切术,于术后3年死于盆腔转移;2例分别于术后8个月和术后3年死于远处转移;7例随访至今,未出现复发及转移。1年生存率为83.3%（10/12）,5年生存率为37.5%（3/8）。结论脐尿管癌是一种少见的高恶性肿瘤,发病率低,临床表现隐匿,预后极差。扩大性膀胱部分切除术为治疗脐尿管癌的主要术式。进行盆腔淋巴结清扫对肿瘤预后判断及辅助治疗选择有一定临床意义。根据病理及复发情况,可行化疗或积极补救性手术,以期延长患者生存期。Objective To explore the clinical characteristics and curative effect of urachal carcinoma,and to improve the diagnosis and treatment of the disease. Methods The clinical data,therapy and outcomes of 15 patients diagnosed as urachal carcinoma were retrospectively reviewed and analyzed. Results Pathological diagnosis were 7 cases of mucinous adenocarcinoma,6 cases of simple adenocarcinoma,1 case of mucinous adenocarcinoma associated with transitional cell carcinoma,1 case of small cell neuroendocrine carcinoma.Sheldon clinical stage：1 case of stageⅡ,9 cases of stageⅢ A,4 cases of stage Ⅲ B and 1 case of Ⅲ C.14 cases were treated by extended partial cystectomy with umbilical resection,including one case underwent pelvic lymph node dissection,and one case was treated with partial cystectomy.12 cases were followed up from 3to 67 months with a median of 36 months.The small cell neuroendocrine carcinoma was given MEP chemotherapy,and died within 3 mouths.Of 15 cases,2 cases received transurethral resection of bladder tumor for bladder recurrence at 12 mouths and 15 mouths of post-operation respectively,and died form metastasis at 1.5years and 3 years after primary operation.Two cases died from extensive metastasis at 8 mouths and 3 years post-operation.The 1-year survival rate was 83.3%（10/12）,and the 5-year survival rate was 37.5%（3/8）.7 patients are alive without recurrence and metastasis.10 patients had a long history of smoking. Conclusions Urachal carcinoma is a rare but highly malignant epithelial cancer with poor prognosis.The exposure to tobacco may be a risk factor for urachal carcinoma.Extended partial cystectomy is main treatment and maybe combined with chemotherapy and salvage surgery according to the pathological stage and recurrence for the aim of survival improvement.

关 键 词：脐尿管癌 诊断 治疗 

分 类 号：R737.1[医药卫生—肿瘤]