脐尿管癌临床病理特点分析

目的：探讨脐尿管癌的临床病理特点、诊断和预后。方法回顾性分析2003年6月至2015年5月梅州市人民医院病理诊断为脐尿管癌的11例患者的临床病理资料。结果11例脐尿管癌患者中男性8例，女性3例，年龄20~65岁，平均45岁。肉眼血尿为主要症状10例(其中2例伴黏液尿)，下腹部疼痛1例。肿瘤均位于膀胱顶壁或前壁。组织学类型包括黏液腺癌5例，腺神经内分泌癌1例，肠型腺癌4例，印戒细胞癌1例。4例镜下见脐尿管剩余结构。腺癌均表达CK20和CDX-2，部分病例不同程度表达CK7和34BE12。腺神经内分泌癌还表达CD56、P16和CgA。Sheldon分期Ⅱ期2例，Ⅲa期9例。7例患者术后随访2~65个月，1例术后9个月死于腹腔广泛转移，1例术后23个月出现肺转移，余5例未见复发和转移。结论脐尿管癌是罕见肿瘤，发现时常分期较晚。病理诊断需结合肿瘤部位，免疫组化染色在脐尿管腺癌、膀胱腺癌和转移性结直肠腺癌的鉴别诊断作用有限，但有助于神经内分泌癌诊断。

Clinicopathologic analysis of urachal carcinoma

Objective To explore the clinicopathological findings, diagnosis and prognosis of urachal carcino-ma. Methods The clinical data from 11 patients of urachal carcinoma diagnosed in our hospital from Jun. 2003 to May 2015 were retrospectively reviewed. Results Among the 11 patients, there were 8 males and 3 females, with the mean age of 45 years (20~65 years). The major symptom was gross hematuria for 10 patients and pain in lower abdomen for 1 patient. Tumor was located in the dome/anterior wall of bladder. Histologic subtype included 5 cases of mucinous adeno-carcinoma, 1 case of adenoneuroendocrine carcinoma, 4 cases of enteric type adenocarcinoma and 1 case of signet ring cell carcinoma. Urachal remnants were identified in 4 cases. Adenocarcinoma was positive for CK20 and CDX-2 and variably positive for CK7 and 34BE12. Adenoneuroendocrine carcinoma was positive for CD56, P16 and CgA too. Shel-don stage wasⅡin 2 patients andⅢa in 9 patients. Seven patients were followed up for 2~65 months, 1 of which died of peritoneum metastasis 9 months after operation, 1 had lung metastasis 23 months after operation, and 5 showed no re-currence and metastasis. Conclusion Urachal carcinoma is a rare tumor that presents with higher clinical stage. Patho-logical diagnosis should be made according to the location of tumor. Immunostains do not unequivocally discriminate be-tween urachal adenocarcinoma, bladder adenocarcinoma and colorectic adenocarcinoma, but is useful for the diagnosis of neuroendocrine carcinoma.