Indiana University, Department of Pathology and Laboratory Medicine, Indianapolis, IN 46202.
Indiana University, Department of Pathology and Laboratory Medicine, Indianapolis, IN 46202.
Hum Pathol. 2019 Aug;90:27-36. doi: 10.1016/j.humpath.2019.04.012. Epub 2019 May 3.
Accurate diagnosis of plasmacytoid urothelial carcinoma (PUC) is important given its poor prognosis and frequent presentation at high stage. We aim to assess the clinicopathological features, molecular aberrations, and follow-up data in a series of PUC cases from a single tertiary cancer center. Seventy-two urinary bladder, ureteral, and renal pelvic specimens with urothelial carcinoma with plasmacytoid differentiation were identified. Immunohistochemical stains were performed on 48 cases. Among urinary bladder origin markers, GATA3 was most sensitive (96%). Breast carcinoma markers (estrogen receptor, mammaglobin) were usually negative, but progesterone receptor stained 1 case (4%). Neuroendocrine markers CD56 and TTF-1 were each positive in 1 case (4% and 4%, respectively). Gastrointestinal adenocarcinoma marker CDX2 was positive in 4 cases (15%), but nuclear β-catenin was negative in all cases. CD138 was positive in 83% and E-cadherin expression was lost in 57% of cases. Fluorescence in situ hybridization using the UroVysion Bladder Cancer Kit and FGFR3 mutational analysis using polymerase chain reaction were performed on 15 cases; deletion of chromosome 9p21 was common (60%), and FGFR3 mutations were detected in 60% of cases (5 cases had both deletion 9p21 and FGFR3 mutations). Cases were divided into 3 morphologic groups: classic (29%), desmoplastic (35%), and pleomorphic (36%). The 3 morphologic subtypes had distinct survival outcomes (P = .083), with median survival for all patients 18 being months versus 10 months for the desmoplastic group.
准确诊断浆母细胞性尿路上皮癌（PUC）很重要，因为其预后较差，且常表现为晚期。我们旨在评估单一三级癌症中心一系列 PUC 病例的临床病理特征、分子异常和随访数据。确定了 72 例具有浆母细胞分化的尿路上皮癌的膀胱、输尿管和肾盂标本。对 48 例病例进行了免疫组织化学染色。在尿路上皮来源标志物中，GATA3 的敏感性最高（96%）。乳腺癌标志物（雌激素受体、乳球蛋白）通常为阴性，但孕激素受体染色 1 例（4%）。神经内分泌标志物 CD56 和 TTF-1 分别在 1 例（4%和 4%）中呈阳性。4 例（15%）胃肠道腺癌标志物 CDX2 阳性，但所有病例核β-连环蛋白均为阴性。CD138 阳性率为 83%，E-钙黏蛋白表达缺失率为 57%。对 15 例病例进行了 UroVysion 膀胱癌试剂盒荧光原位杂交和 FGFR3 突变分析用聚合酶链反应；9p21 染色体缺失常见（60%），60%的病例中检测到 FGFR3 突变（5 例同时存在 9p21 缺失和 FGFR3 突变）。病例分为 3 种形态学组：经典型（29%）、纤维母细胞型（35%）和多形性（36%）。3 种形态亚型的生存结果明显不同（P=0.083），所有患者的中位生存时间为 18 个月，纤维母细胞型为 10 个月。
