Urachal carcinoma: a clinicopathologic analysis of 24 cases with outcome correlation.
Urachal carcinoma: a clinicopathologic analysis of 24 cases with outcome correlation.
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DOI：
10.1097/pas.0b013e31819aa4ae
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发表时间：
2009-05
期刊：
影响因子：
--
通讯作者：
Olgac S
中科院分区：
文献类型：
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作者：
Gopalan A;Sharp DS;Fine SW;Tickoo SK;Herr HW;Reuter VE;Olgac S
脐尿管癌主要发生在膀胱穹隆，占膀胱腺癌的22%至35%，通常通过部分膀胱切除术并整块切除正中脐韧带和脐来治疗。与临床结果相关的详细病理学研究很少。我们回顾了从1984年至2005年在我们机构诊断和治疗的67例圆顶型肿瘤数据库中选择的24例病例的组织学资料和临床资料。所有24例患者的随访信息均可用。诊断时的平均年龄为52岁（范围26-68）。男性15例，女性9例。23例发生于室顶，1例发生于室顶和前壁。13例为单纯腺癌，NOS，9例为肠型腺癌，2例为腺癌伴淋巴上皮瘤样癌和尿路上皮癌伴胞浆透明灶。2例可见局灶性印戒细胞特征。膀胱炎4例，腺性膀胱炎2例。在除1例外的所有病例中，囊性/腺性膀胱炎均为局灶性，主要位于膀胱内，覆盖脐尿管肿瘤。脐尿管残留15例，其中良性尿路上皮型6例，腺瘤型9例。3例膀胱尿路上皮癌。在所有三个，脐尿管残余物被确定，并显示从良性腺瘤上皮的过渡。在免疫组化上，这些肿瘤对CK 20呈阳性，对CK 7和34 BE 12呈阴性。大多数显示β-连环蛋白的胞浆膜染色模式，尽管在1例病例中，确定了局灶性核免疫反应性。谢尔顿病理分期：pT 1 0例，pT 2 2例，pT 3a 8例，pT 3b 11例，pT 3c 1例，pT 4a 1例，pT 4 b 1例。1例患者软组织切缘阳性。平均随访时间为40个月（范围0.3-157.6）。24例中7例（29%）局部复发。单纯行部分膀胱切除术的患者局部复发率（37.5%）高于行根治性手术的患者（27%）。9例（37.5%）患者发生远处转移，其中4例既往无局部复发。7例患者（29%）死于该病。所有局部复发和转移的病例均为pT 3或更高分期。病理分期是脐尿管癌的重要预后因素。膀胱癌累及表面尿路上皮和腺性膀胱炎不一定排除脐尿管癌的诊断。免疫染色不能明确区分脐尿管癌和结直肠癌，但34 BE 12的弥漫阳性将支持脐尿管癌的诊断，β连环蛋白的弥漫核免疫反应性将不利于脐尿管癌的诊断。局部复发可能是由于远端尿路上皮内的种植，特别是在息肉样结构的肿瘤中，并且开口进入膀胱腔。尽管切缘阴性，但手术类型可能对局部复发有影响。
Urachal carcinomas occur mostly in the bladder dome, comprising 22 to 35% of vesical adenocarcinomas, and are generally treated by partial cystectomy with en bloc resection of the median umbilical ligament and umbilicus. Detailed pathologic studies with clinical outcome correlation are few. We reviewed histologic material and clinical data from 24 cases selected from a database of 67 dome-based tumors diagnosed and treated at our institution from 1984 to 2005. Follow-up information was available for all 24 patients. The mean age at diagnosis was 52 years (range 26-68). 15 patients were male and 9 were female. Location was the dome in 23, and dome and anterior wall in 1. Thirteen cases were pure adenocarcinoma, NOS, 9 were enteric type adenocarcinoma and 2 were adenocarcinoma with focal components of lymphoepithelioma-like carcinoma and urothelial carcinoma with cytoplasmic clearing. Signet ring cell features were focally seen in 2 cases. Cystitis cystica and cystitis glandularis were seen in 4 and 2 cases, respectively. In all instances but one, cystitis cystica/ glandularis was focal and predominantly in the bladder overlying the urachal neoplasm. Urachal remnants were identified in 15 cases: the urachal epithelium was benign urothelial-type in 6 cases and showed adenomatous changes in 9. The overlying bladder urothelium was colonized by adenocarcinoma in 3 cases. In all three, urachal remnants were identified and showed transition from benign to adenomatous epithelium. On immunohistochemistry, these tumors were positive for CK20 and variably positive for CK7 and 34BE12. The majority showed a cytoplasmic membranous staining pattern for beta-catenin, although in 1 case, focal nuclear immunoreactivity was identified.. The Sheldon pathologic stage was pT1 in 0, pT2 in 2, pT3a in 8, pT3b in 11, pT3c in 1, pT4a in 1 and pT4b in 1 patient. One patient had a positive soft tissue margin. The mean follow-up period was 40 months (range 0.3-157.6). Seven of 24 (29%) cases recurred locally. The incidence of local recurrence was higher in patients who underwent a partial cystectomy alone (37.5%) versus those who had a more radical surgery (27%). Distant metastases occurred in 9 (37.5%) patients, 4 of which had no prior local recurrence. Seven patients (29%) died of the disease. All cases with locally recurrent and metastatic disease belonged to stage pT3 or higher. Pathologic stage is an important prognostic factor in urachal carcinoma. Surface urothelial involvement by carcinoma and presence of cystitis cystica/ glandularis do not necessarily exclude the diagnosis of urachal carcinoma. Immunostains do not unequivocally discriminate a urachal from a colorectal carcinoma, but diffuse positivity for 34BE12 would support, and diffuse nuclear immunoreactivity for beta catenin would militate against, a diagnosis of urachal carcinoma.. Local recurrence may be due to seeding within the distal urothelial tract, particularly in tumors with a configuration that is polypoid and which open into the bladder cavity. The type of surgery performed may have an effect on local recurrence despite negative margins of resection.