膀胱小细胞神经内分泌癌的病理及临床特征

Clinical and pathological characterization of small cell neuroendocrine carcinoma in urinary bladder

目的探讨膀胱小细胞神经内分泌癌的临床病理特征,提高对该病的认识和诊治水平。方法介绍本例的病理与临床特征,结合国内已报道的39例临床资料及文献,分析该肿瘤的组织来源、病理和免疫组化特征、诊治及预后。结果本例行经尿道膀胱肿瘤电切除,术后病理诊断为小细胞神经内分泌癌。免疫组化染色示肿瘤细胞均表达神经内分泌标记物NSE、CD56和Syn。行膀胱全切术及化疗后,随访1年未见复发。结论膀胱小细胞神经内分泌癌是一种比较少见的高度恶性肿瘤,有独特的病理形态。临床表现以血尿为主要症状,早期即可发生转移,预后凶险。确诊依靠病理检查及免疫组化,手术切除联合放疗和化疗是主要的有效治疗手段。早期发现是改善预后的关键。

Objective To study the clinical and pathological characteristics of bladder small cell neuroendocrine carcinomas （BSNEC） and to improve the diagnosis and treatment level of this kind of diseases. Methods The pathological and clinical characterizations of this case were reported, and then the histogenesis, the pathological and immunohistochemical characteristics, the diagnosis and prognosis of BSNEC were analyzed by summarizing the clinical data of related 39 cases in the Chinese literatures. Results The transurethral resection of bladder tumor were performed. The pathological diagnosis was BSNEC prostoperationally and immunohistochemical examination showed that the tumors were stained for neuroendocrine markers such as neuron-specific enolase （NSE）, CD56 and synaptophysin （ Syn）. The patient underwent radical cystectomy and chemotherapy again. Follow-up of one year showed no recurrence. Conclusions BSNEC is a rare and highly malignant bladder cancer, which has specific pathological characteristics. Hematuria is a chief symptom of this carcinomas. Metastasis happens earlier and the prognosis is poor. Final diagnosis depends on pathology and immunohistochemistry. Surgical resection combined with radiotherapy and chemotherapy is the best available therapy. The key to improve the prognosis is its early detection.