出 处:《现代泌尿生殖肿瘤杂志》2018年第1期16-21,共6页Journal of Contemporary Urologic and Reproductive Oncology
摘 要:目的总结并探讨原发性膀胱浆细胞样/印戒细胞尿路上皮癌的临床特征和治疗预后,提高对该疾病的认识和诊疗水平。方法结合我院曾收治的1例和国内1986~2017年个案报道的44例原发性膀胱浆细胞样/印戒细胞尿路上皮癌的临床资料,回顾性分析其发病机制、病理特点、临床表现、诊疗方案、随访预后等。结果 45例中,肿瘤好发于顶壁31例(68.9%);单纯性印戒细胞癌37例(82.2%),混合性印戒细胞癌8例(17.8%);TNM分期,T2期以上为37例(82.2%),其中T3期和T4期26例(57.8%);手术病例41例(91.1%),其中采用经尿道膀胱肿瘤电切术3例(7.3%),膀胱部分切除术17例(41.5%),根治性膀胱全切术21例(51.2%);15例(36.6%)术后未行任何辅助治疗,行膀胱灌注化疗5例(12.2%),全身化疗13例(31.7%),全身化疗+膀胱灌注化疗5例(12.2%),全身化疗+放疗3例(7.3%)。失访6例(13.3%);随访时间≤1年的28例(62.2%),其中15例在1年内死亡,2例复发;1年<随访时间≤2年的8例(17.8%),其中2例在13个月时死亡;生存时间>2年的只有3例(6.7%)。生存期最短的为2个月,最长的为44个月无复发,平均生存时间约25.1个月,中位生存时间约13个月。结论原发性膀胱浆细胞样/印戒细胞尿路上皮癌临床罕见、恶性程度高、进展快、早期诊断困难、预后差,早期行根治性膀胱全切术结合术后辅助放化疗,可改善其治疗效果,但整体预后差。Objective To investigate the clinical characteristics and treatment method as well as prognosis of primary plasmacytoid/signet-ring cell urothelial carcinoma of the bladder,and to improve the level of recognition,diagnosis and treatment on disease. Methods Individual patient's data were obtained from 1 case treated by us and 44 cases of domestic case reports(1986-2017),a total of 45 cases of primary plasmacytoid/signet-ring cell urothelial carcinoma of the bladder were analysised retrospectively,including its pathogenesis,pathology characteristics,clinical features,diagnosis,treatment and prognosis of follow up. Results In 45 cases,31 cases(68.9%)were found tumor in upper wall;37 cases(82.2%)in simple type and 8 cases(17.8%)in mixed type.TNM staging,T 2 accounted for the vast majority of which,were 37 cases(82.2%),T 3 and T 4 accounted for 26 cases(57.8%);surgical cases were 41 cases(91.1%),in which 3 cases(7.3%)adopted TURBT,17 cases(41.5%)adopted partial cystectomy,21 cases(51.2%)adopted radical cystectomy;15 cases(36.6%)weren't given any postoperative adjuvant therapy,5 cases(12.2%)were given bladder perfusion chemotherapy,13 cases(31.7%)were given systemic chemotherapy,5 cases(12.2%)were given systemic chemotherapy+bladder perfusion chemotherapy,3 cases(7.3%)were given systemic chemotherapy+radiotherapy.Six cases(13.3%)weren’t followed up,and 28 cases(62.2%)were followed up for≤1 year.Fifteen cases of which died within 1 year,2 cases recurred.Eight cases(17.8%)were followed up for 1 to 2 year,2 cases died in the 13 months,only 3[HK]cases(6.7%)were followed up and survived for>2 years.The shortest lifetime was 2 months,and the longest was 44 months without recurrence.The mean survival time was about 25.1 months,and the median survival time was about 13 months. Conclusions Primary plasmacytoid/signet-ring cell urothelial carcinoma of the bladder is a rare tumor with high malignancy,fast progress,difficult early diagnosis and poor prognosis.Radical cystectomy in the early combined with postoperative radiothe
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