脐尿管腺癌13例临床病理分析

Urachal adenocarcinoma: a clinicopathological analysis of 13 cases and review of literature

目的探讨脐尿管腺癌的临床病理特点、鉴别诊断、治疗及预后。方法收集13例脐尿管腺癌病例,结合临床辅助检查、组织学及免疫组化特征进行分析。结果13例患者中,男性11例,女性2例,年龄28~79岁,平均51岁。肿瘤位于膀胱顶部、左侧壁、左前壁、前壁正中,最大径2~7 cm;组织学类型,脐尿管黏液腺癌6例,非特殊类型腺癌4例,肠型腺癌2例,黏液腺癌伴印戒细胞癌1例。免疫组化:CK7+/-,CK20、CDX2弥漫强(+),瘤细胞膜β-Cantenin弥漫强(+);p63、GATA-3(-)。结论脐尿管腺癌是一种罕见但高度恶性的肿瘤,在膀胱顶部或前壁的肌层见浸润型腺癌并伴有血尿时,应高度怀疑是否存在脐尿管腺癌。目前治疗采取膀胱部分切除加脐尿管切除,辅助全身化疗以提高患者生存率。

Objective To investigate the clinicopathological features,differential diagnosis,treatment and prognosis of urachal adenocarcinoma.Methods Thirteen cases of urachal adenocarcinoma were collected and analyzed with clinical auxiliary examination.Results Among the 13 patients,11 were male and 2 were female,aged 28-79 years,with an average age of 51 years.The maximum diameter of the tumors was 2-7 cm.The tumors were located at the top of the bladder,the left lateral wall,the left anterior wall and the middle of the anterior wall,respectively.Histological types included urachal mucinous adenocarcinoma in 6 cases,non-specific adenocarcinoma in 4 cases,intestinal adenocarcinoma in 2 cases,mucinous adenocarcinoma with signet ring cell carcinoma in 1 case.Immunohistochemistry showed that the tumor was partially positive for CK7,diffuse and strongly positive for CK20,CDX2 and beta-Cantenin,but negative for P63 and GATA-3.Conclusion Urachal adenocarcinoma is a rare but highly malignant neoplasm.When invasive adenocarcinoma with hematuria is seen in the muscular layer of the top or anterior wall of the bladder,the presence of urachal adenocarcinoma should be highly suspected.At present,partial cystectomy combined with urachal resection and systemic chemotherapy are recommended to improve the survival rate of patients.