泌尿系统神经内分泌肿瘤16例临床病理分析

Neuroendocrine tumors in the urinary system:a clinicopathological analysis of 16 cases

目的探讨泌尿系统神经内分泌肿瘤(neuroendocrine tumors,NETs)的临床病理学特征、诊断及鉴别诊断、治疗及预后。方法回顾性分析16例泌尿系统NETs(膀胱11例、输尿管1例、肾脏4例)的临床病理特点、免疫表型、诊断及鉴别诊断、治疗及预后,并复习相关文献。结果 16例NETs中男性11例,女性5例;年龄48～84岁,平龄65岁;小细胞癌10例,副神经节瘤4例,类癌及不典型类癌各1例;12例患者有随访资料,其中6例死亡,均为小细胞癌(3～21个月),6例存活,包括4例膀胱副神经节瘤(7～88个月)、1例肾类癌(14个月)和1例膀胱小细胞癌合并尿路上皮癌(42个月)。结论 NETS发生在泌尿系统中较为罕见,主要为小细胞癌、副神经节瘤及类癌。根据其典型组织形态及神经内分泌免疫组化标志物(Syn、CgA、CD56)阳性鉴别,需谨慎排除转移可能。副神经节瘤及类癌预后较好,小细胞癌侵袭性高,预后差。

Purpose To investigate the clinicopathological characteristics, diagnosis, differential diagnosis, treatment and prognosis of urinary system neuroendocrine tumors（ NETs）.Methods 16 cases of NETs of the urinary system（11 cases of bladder, 1 case of ureter and 4 cases of kidney） were collected to analyze retrospectively the clinicopathological features, immunohistochemical markers, diagnosis and differential diagnosis,treatment and prognosis with review of the literature. Results There were 11 males and 5 females with mean age 65-year-old（48 -84 years）. Ten cases were small cell carcinoma, four cases were paraganglioma, one typical carcinoid and one atypical carcinoid. 12 cases were followed up, of which 6 cases were dead（all were small cell carcinomas, 3-21 months） and 6 cases were survived, including 4 cases of bladder paraganglioma（7 -88 months）, one renal carcinoid（ 14 months） and one bladder small cell carcinoma with urothelial carcinoma（42 months）. Conclusion NETs occurred in the urinary system are extremely rare. The common types are small cell carcinoma,paraganglioma and carcinoid tumor. Definite diagnosis should be made by depending on characteristic morphology and immunohistochemistry（neuroendocrine markers Syn, CgA, CD56 are positive）,and rule out metastasis carefully. Paraganglioma and carcinoid tumors are associated with favorable clinical outcomes and small cell carcinoma is associated with poor prognosis because of its high aggression.