背景:微囊性尿路上皮癌(MUC)是一种罕见的变异型尿路上皮癌,其组织学表现类似于病变开始。到目前为止,报告了大约50例。这里,我们调查了MUC的临床病理特征。
方法:收集临床数据和石蜡包埋的组织块。免疫组化染色和聚合酶链反应-Sanger测序检测MUC的表型和TERT突变状态,分别。
结果:患者平均年龄为58.8±14.5岁,以男性为主(8:2)。1例病理分期为T1,T2在三种情况下,T3在四种情况下,和T4两种情况。在1-3年内随访的所有5例患者均发生肿瘤转移或死亡。组织学分析显示微囊,管状,cribriform,偶尔会有绳状结构,通常缺乏间质反应。流明是空的,含有嗜酸性粒细胞分泌,或者充满了粘蛋白。微囊/小管/筛状图案由平面排列,长方体,图章戒指,或柱状类型的上皮。长方体,图章戒指,柱状类型代表尿路上皮癌的“腺化生”或腺分化。免疫组织化学分析揭示了涉及腔标记FOXA1和GATA3以及基础标记CK5/6和CD44的不同共表达模式。根据GATA3+/CK14-标准,所有10例患者均表现为管腔表型,而根据FOXA1+/CK14-标准,有9例表现出腔表型。7例检测到端粒酶逆转录酶C228T突变。
结论:MUC是一种罕见的变种,具有一种看似良性的尿路上皮癌,通常被认为是晚期肿瘤,预后不良。它表现出明显的腔和基底标志物的共表达,以及TERT-C228T突变。
BACKGROUND: Microcystic urothelial carcinoma (MUC) is a rare variant of urothelial carcinoma with histological appearances similar to begin lesions. Thus far, approximately 50 cases have been reported. Here, we investigated the clinicopathological features of MUC.
METHODS: Clinical data and paraffin-embedded tissue blocks were collected. Immunohistochemical staining and polymerase chain reaction-Sanger sequencing were performed to detect the phenotype and TERT mutation status of MUC, respectively.
RESULTS: The mean patient age was 58.8 ± 14.5 years, with a male predominance (8:2). The pathological stage was T1 in one case, T2 in three cases, T3 in four cases, and T4 in two cases. Tumor metastases or death occurred in all five patients who were followed up within 1-3 years. Histological analyses revealed microcystic, tubular, cribriform, and occasionally cord-like structures, which generally lacked interstitial reactions. The lumens were empty, contained eosinophilic secretion, or were filled with mucin. The microcysts/tubules/cribriform patterns were lined by flat, cuboid, signet ring, or columnar types of epithelia. The cuboid, signet ring, and columnar types represented \"glandular metaplasia\" or glandular differentiation of urothelial carcinoma. Immunohistochemistry analyses revealed distinct co-expression patterns involving the
luminal markers FOXA1 and GATA3, as well as the basal markers CK5/6 and CD44. All 10 cases exhibited a
luminal phenotype according to the GATA3+/CK14- criterion, whereas nine cases exhibited a
luminal phenotype according to the FOXA1+/CK14- criterion. The telomerase reverse transcriptase-C228T mutation was detected in seven cases.
CONCLUSIONS: MUC is a rare variant with a deceptively benign form of urothelial carcinoma, which is generally identified as a late-stage tumor with a poor prognosis. It exhibits distinct co-expression of
luminal and basal markers, along with the TERT-C228T mutation.