膀胱副神经节瘤16例临床病理分析

Paraganglioma of the bladder:a clinicopathological analysis of 16 cases

目的 探讨膀胱副神经节瘤(paraganglioma of the urinary bladder,PUB)的临床病理特点、诊断、鉴别诊断及预后的关系。方法 收集16例PUB的临床病理资料,采用免疫组化EnVision两步法检测CD56、Syn、CgA、CK、CK7、CK20、GATA-3、SDHB、S-100和Ki-67等的表达,分析其与病理特征的关系,并复习相关文献。结果 16例PUB中患者年龄29~73岁,中位年龄53岁。10例为体检发现,6例表现为腰腹疼痛、间断性血尿或排尿终末腹痛,1例术中出现血压骤升。肿瘤最大径0.5~4 cm,平均直径3.5 cm。镜下见肿瘤位于黏膜下层或固有肌层,呈结节状或浸润性生长,未见包膜。肿瘤细胞呈巢状(Zellballen)、器官样排列,细胞巢间有血管网分隔。瘤细胞圆形或卵圆形,胞质丰富,嗜酸性或嫌色性,部分病例胞质内见黑色素样色素,核呈圆形,未见明显核仁,核分裂象罕见。免疫表型:肿瘤细胞弥漫表达神经内分泌标志物CD56、Syn、CgA,不表达CK、CK7、CK20,部分病例表达尿路上皮标记GATA-3,而SDHB均表达;Ki-67增殖指数为1%~10%。随访14例患者均未见转移,有1例局部复发,行一代测序检测提示FH基因体细胞突变。结论 PUB临床多以体检发现,提高对PUB的认识可避免出现术中高血压危象;其易与浸润性尿路上皮癌混淆,需结合免疫组化做出正确诊断。

Purpose To investigate the clinicopathological features,diagnosis,differential diagnosis and prognostic of paraganglioma of the urinary bladder(PUB).Methods The clinicopathological data of 16 cases of PUB were collected.The expressions of CD56,Syn,CgA,CK,CK7,CK20,GATA-3,SDHB,S-100 and Ki-67 were detected by immunohistochemical staining with EnVision two-step method.The relationship between them and pathological features was analyzed,and the relevant literatures were reviewed.Results The age of 16 PUB cases ranged from 29 to 73 years(median,53 years).10 cases were found in physical examination,6 cases presented with lumbar and abdominal pain,or intermittent hematuria,or abdominal pain at the end of urination.1 case occurred intraoperative hypertensive crisis.Grossly,the tumor in size ranged from 0.5 to 4 cm in the maximum diameter(mean 3.5 cm).Microscopically,the tumor located in the submucosa or muscular wall with invasive growth,and showed the typical “Zellballen” growth pattern by prominent vascular network.The cells were round or oval with abundant eosinophilic or chromophobe cytoplasm,which may contain pigmented granules.The nuclei were round with small nucleoli.Immunohistochemically,the tumor cells were positive for CD56,Syn and CgA,but negative for CK,CK7 and CK20.GATA-3 was expressed in some cases and SDHB was positive.Ki-67 index was 1%-10%.No metastasis was observed in 14 patients at follow-up,and 1 patient suffered from local recurrence who had somatic mutation of the FH gene with 1 st generation sequencing test.Conclusion PUB is mostly found by physical examination.Improving the understanding of PUB can avoid intraoperative hypertensive crisis.It is easy to be confused with invasive urothelial carcinoma,which needs to be diagnosed correctly combined with immunohistochemistry.