15例脐尿管癌的临床特征及预后分析

柯春锦; 杨春光; 曾星; 甘家骅; 田继华; 沈源清; 李宋; 李乐; 胡志全

doi:10.13201/j.issn.1001-1420.2020.04.012

15例脐尿管癌的临床特征及预后分析

- 华中科技大学同济医学院附属同济医院泌尿外科(武汉, 430030)

详细信息

通讯作者: 胡志全,E-mail:huzhiquan2000@163.com

中图分类号: R737

收稿日期: 2019-07-26

Clinical features and prognostic analysis of 15 patients with urachal carcinoma

- Department of Urology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China

More Information

Corresponding author: HU Zhiquan, E-mail: huzhiquan2000@163.com

Received Date: 26 July 2019

摘要

摘要: 目的:分析脐尿管癌患者的临床及预后特征,提高临床医生对脐尿管癌的诊疗水平。方法:回顾性分析我院2012年5月~2019年5月收治的15例脐尿管癌患者的临床资料、治疗方案以及随访结果,所需资料主要通过追踪病历及电话访问获得,运用SPSS 17.0进行统计分析并计算生存率。结果:脐尿管癌患者共有15例,其中男13例,女2例。中位年龄46岁,平均年龄44.1(25~65)岁。最常见的首发症状为肉眼血尿,其次是尿路刺激症状、腹痛。15例均为腺癌,其中中分化腺癌6例,中低分化腺癌3例,印戒细胞癌1例,黏液腺癌1例,混合型腺癌1例,未分型3例。免疫组化中CK20、CDX-2均可呈弥漫性强阳性,CK7为60%阳性率,GATA-3、PSA阴性。膀胱镜或影像学显示肿块组织均处于膀胱顶壁或前壁或沿膀胱中线分布。依据Sheldon分期,Ⅲa期11例(73.3%),Ⅲc期1例(6.7%),Ⅳb期2例(13.3%),1例(6.7%)由于外院术后转入,无具体分期资料。本中心患者大部分是采用手术治疗结合化疗或单纯手术治疗,15例患者当中多数接受扩大的膀胱部分切除术,切除范围包括脐及脐尿管,还有耻骨后间隙的结缔组织,以及肿瘤表面腹膜,2例Ⅳb晚期患者均未行手术而给予姑息性化疗。随访时间1~70个月,有效随访为10例,失访5例,中位随访时间为14个月,平均随访时间为19个月,中位生存时间70个月,1年及5年生存率分别为66%、53%。结论:脐尿管癌发病隐匿,最常见的临床症状为肉眼血尿,早期临床症状多不典型,当出现临床症状时多为Ⅲ期及以上,预后较差。扩大的膀胱部分切除术与根治性膀胱切除术生存结局相似,膀胱容量保留充分时能提高患者的生活质量,术后辅助化疗或放疗有希望进一步改善预后。对于远处转移患者,可给予化疗或靶向治疗等全身性治疗方案。
- 脐尿管癌 /
- 诊断 /
- 治疗 /
- 化疗 /
- 预后
Abstract: Objective: To analyze the clinical and prognostic features of patients with urachal carcinoma, and to improve the diagnosis and treatment of urachal carcinoma. Method: The clinical data, treatment plan and follow-up results of 15 patients with urachal carcinoma treated in our hospital from May 2012 to May 2019 were retrospectively analyzed. The required data were mainly obtained through tracking medical records and telephone interviews. Statistical analysis and survival rate were calculated using SPSS 17.0. Result: There were 15 patients with urachal carcinoma, including 13 males and 2 females. The median age is 46 years, with an average age of 44.1(range, 25-65) years. The most common initial symptom is gross hematuria, followed by urinary tract irritation and abdominal pain. Fifteen cases were adenocarcinoma, including 6 cases of moderately differentiated adenocarcinoma, 3 cases of moderately to poorly differentiated adenocarcinoma, 1 case of signet ring cell carcinoma, 1 case of mucinous adenocarcinoma, 1 case of mixed adenocarcinoma, and 3 cases of undifferentiated type. In immunohistochemistry, both CK20 and CDX-2 can be diffusely and strongly positive, with 60% positive rate for CK7 and negative GATA-3 and PSA. Cystoscopy or imaging showed that the mass tissue was located in the dome or anterior wall of the bladder or along the midline of the bladder. According to Sheldon staging, 11 cases(73.3%) in stage Ⅲa, 1 case(6.7%) in stage Ⅲc, 2 cases(13.3%) in stage Ⅳb. The rest 1 case(6.7%) was transferred from other hospital postoperatively, so Sheldon staging was unknown. Most of the patients in our center were treated with surgery combined with chemotherapy or surgery alone. Most of the 15 patients underwent extended partial cystectomy, including the umbilicus and urachus, as well as the connective tissue in the retropubic space and the peritoneum on the tumor surface. Palliative chemotherapy was given to 2 patients with advanced Ⅳb disease without surgery. The follow-up time ranged from 1 to 70 months, with effective follow-up of 10 cases and loss of follow-up of 5 cases. The median follow-up time was 14 months, the mean follow-up time was 19 months, and the median survival time was 70 months. One-year and 5-year survival rates were 66% and 53%, respectively.Conclusion: The incidence of urachal carcinoma is concealed. The most common clinical symptom is gross hematuria. The early clinical symptoms are atypical. When clinical symptoms appear, they are mostly stage Ⅲ or above with poor prognosis. Extensive partial cystectomy is similar to radical cystectomy in the survival outcome. When the bladder volume is adequately preserved, the patient's quality of life can be improved. Postoperative adjuvant chemotherapy or radiotherapy may hope to further improve the prognosis. For patients with distant metastases, systemic treatments such as chemotherapy or targeted therapy can be given.
- urachal carcinoma /
- diagnosis /
- treatment /
- chemotherapy /
- prognosis

HTML全文

参考文献(19)

[1]	Wilcox DT,Godbole PP,Koyle MA.Pediatric urology:Surgical complications and management[M].BK:Blackwell Publishing Ltd,2008:92-97.
[2]	Scabini S,Rimini E,Romairone E,et al.Urachal tumour:case report of a poorly understood carcinoma[J].World J Surg Oncol,2009,7:82.
[3]	Gopalan A,Sharp DS,Fine SW,et al.Urachal carcinoma:a clinicopathologic analysis of 24 cases with outcome correlation[J].Am J Surg Pathol,2009,33(5):659-668.
[4]	Johnson DE,Hodge GB,Abdul-Karim FW,et al.Urachal carcinoma[J].Urology,1985,26:218-221.
[5]	Siefker-Radtke A.Urachal adenocarcinoma:a clinician's guide for treatment[J].Seminars Oncol,2012,39(5):619-624.
[6]	Wheeler JD,Hill WT.Adenocarcinoma involving the urinary bladder[J].Cancer,1954,7(1):119-135.
[7]	Yu JS,Kim KW,Lee HJ,et al.Uraehal remnant diseases:spectrum of CT and US findings[J].Radiographics,2001,21(2):451-461.
[8]	Paner GP,McKenney JK,Barkan GA,et al.Immunohistochemical analysis in a morphologic spectrum of urachal epithelial neoplasms:diagnostic implications and pitfalls[J].Am J Surg Pathol,2011,35(6):787-798.
[9]	Riva G,Mian C,Luchini C,et al.Urachal carcinoma:from gross specimen to morphologic,immunohistochemical,and molecular analysis.[J].Virchows Archiv,2019,474(1):13-20.
[10]	Siefker-Radtke AO,Gee J,Shen Y,et al.Multimodality management of urachal carcinoma:the M.D.Anderson Cancer Center experience[J].J Urol,2003,169(4):1295-1298.
[11]	Ashley RA,Inman BA,Sebo TJ,et al.Urachal carcinoma:clinicopathologic features and long-term outcomes of an aggressive malignancy[J].Cancer,2006,107(4):712-720.
[12]	Bruins HM,Visser O,Ploeg M,et al.The clinical epidemiology of urachal carcinoma:results of a large,population based study[J].J Urol 2012,188(4):1102-1107.
[13]	Pinthus JH,Haddad R,Trachtenberg J,et al.Population based survival data on urachal tumors[J].J Urol,2006,175(6):2042-2047.
[14]	Niedworok C,Panitz M,Szarvas T,et al.Urachal carcinoma of the bladder-impact of clinical and immunohistochemical parameters on patients'prognosis[J].J Urol 2016,195(6):1690-1696.
[15]	Szarvas T,Modos O,Niedworok C,et al.ClinicaI,prognostic,and therapeutic aspects of urachal carcinoma-A comprehensive review with metaanalysis of 1010 cases[J].Urol Oneol Semin&Orig Inves,2016,34(9):388-398.
[16]	Testa I,Verzoni E,Grassi P,et al.Response to targeted therapy in urachal adenocarcinoma[J].Rare Tumors,2014,6(4):5529.
[17]	Goss G,Hirte H,Miller WH,et al.A phase I study of oral ZD 1839 given daily in patients with solid tumors:IND.122,a study of the Investigational New Drug Program of the National Cancer Institute of Canada Clinical Trials Group[J].Invest New Drugs,2005,23(2):147-155.
[18]	Zong L,Chen P.Urachal tumour:case report of a poorly understood carcinoma[J].World J Surg Oncol,2013,11(1):170.
[19]	Reis H,Krafft U,Niedworok C,et al.Biomarkers in Urachal Cancer and Adenocarcinomas in the Bladder:A Comprehensive Review Supplemented by Own Data[J].Dis Markers,2018,2018:7308168.