Wang Zhihua, Lu Tong, Du Lihuan, Hu Zhiquan, Zhuang Qianyuan, Li Youyuan, Wang Cong-Yi, Zhu Hui, Ye Zhangqun
Department of Urology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, 1095 Jiefang Ave., Wuhan, 430030, PR China.
Int J Clin Exp Pathol. 2012;5(6):601-8. Epub 2012 Jul 29.
Plasmacytoid carcinoma of the urinary bladder or plasmacytoid urothelial carcinoma (PUC) is a rare and only recently described histological variant of transitional cell carcinoma (TCC). We herein report the clinical and histopathological features for a new case of PUC. By combining with those reported cases, we intend to define the characteristics of PUC and to provide a therapeutic and prognostic guidance for this disease.
The index case at our institution was a patient with complaint of lower abdominal pain but without any urological symptoms. The patient underwent radical cystectomy, and the representative sections of tumor were submitted for immunohistochemical analysis. The data for this patient were collected from clinical charts, histological review and follow-up studies. We also performed an extensive literature review of PUC including clinical presentation, pathological features, therapy and prognosis.
Clinically, patients with PUC are associated with nonspecific abdominal pain but absent of hematuria. Cystoscopy analysis revealed that PUC is manifested by the coarse and indurated mucosal fold. Macroscopic studies demonstrated an ulcerated firm mass which was present in the left lateral wall of the bladder. Histologically, PUC appeared to be dyscohesive, plasmacytoid cells with eccentric nuclei and abundant eosinophilic cytoplasm with characteristics of plasmacytoid morphology. The tumor cells are negative for E-cadherin, but positive for CD138 expression. This particular patient died 3 months after the radical cystectomy and one course of adjuvant chemotherapy. Literature review revealed that most PUC cases showed similar clinical and pathological features along with poor prognosis.
PUC is a rare tumor associated with poor prognosis due to its advanced clinical stage upon its diagnosis. The delayed diagnosis is mainly due to the late occurrence of hematuria and absence of papulary mucosal surface at cystoscopy. Diagnosis can be achieved based on its typical histological features, clinical history and immunohistochemical results. Other than radical cystectomy, postoperative adjuvant treatment could be a good approach to prolong the survival time of PUC patients.
膀胱浆细胞样癌或浆细胞样尿路上皮癌（PUC）是一种罕见的、最近才被描述的移行细胞癌（TCC）组织学变异类型。我们在此报告一例新的PUC病例的临床和组织病理学特征。通过与已报道病例相结合，我们旨在明确PUC的特征，并为该疾病提供治疗和预后指导。
我们机构的该例索引病例是一名主诉下腹部疼痛但无任何泌尿系统症状的患者。患者接受了根治性膀胱切除术，并将肿瘤的代表性切片送检进行免疫组化分析。该患者的数据来自临床病历、组织学检查和随访研究。我们还对PUC进行了广泛的文献综述，包括临床表现、病理特征、治疗和预后。
临床上，PUC患者伴有非特异性腹痛但无血尿。膀胱镜检查分析显示，PUC表现为粗糙、硬结的黏膜皱襞。大体研究显示膀胱左侧壁有一个溃疡状的坚实肿块。组织学上，PUC表现为细胞间黏附性差、具有偏心核和丰富嗜酸性细胞质的浆细胞样细胞，具有浆细胞样形态特征。肿瘤细胞E-钙黏蛋白阴性，但CD138表达阳性。该特定患者在根治性膀胱切除术后3个月及一个疗程辅助化疗后死亡。文献综述显示，大多数PUC病例表现出相似的临床和病理特征，预后较差。
PUC是一种罕见肿瘤由于其诊断时临床分期较晚预后较差。诊断延迟主要是由于血尿出现较晚以及膀胱镜检查时无乳头状黏膜表面。基于其典型的组织学特征、临床病史和免疫组化结果可实现诊断。除根治性膀胱切除术外，术后辅助治疗可能是延长PUC患者生存时间的良好方法。
