膀胱副神经节瘤12例临床病理分析  

Clinicopathologic analysis Paraganglioma of urinary bladder

作　　者：余春开[1] 宋志刚[2] 

机构地区：[1]首都医科大学附属北京世纪坛医院病理科,北京100038 [2]解放军总医院病理科,北京100853

出　　处：《诊断病理学杂志》2015年第12期744-748,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨膀胱副神经节瘤（PUB）的临床病理学特征、诊断及鉴别诊断。方法研究12例膀胱副神经节瘤病例的临床及病理学特点,并进行免疫组化染色、复习相关文献。结果 12例中男性5例,女性7例,平均发病年龄50.6岁（34~80岁）。肿瘤最大径0.8~4.8 cm。镜下肿瘤组织由主细胞和支持细胞组成,内含丰富的血窦形成的血管网。肿瘤组织在膀胱黏膜下层或肌壁间可表现为穿插性生长,也可膨胀性生长而形成边界清楚的结节。1例肿瘤细胞异型性明显,核分裂象多见,多灶凝固性坏死,局灶可见脉管内瘤栓,Ki-67指数为60%,此例随访88个月,无复发和转移。免疫组化：肿瘤细胞神经内分泌标记物Cg A、Syn和CD56弥漫强（＋）,上皮性标记物（-）,支持细胞S-100（＋）。10例得到随访资料,随访时间4~88个月,均无复发和转移。结论膀胱副神经节瘤临床罕见,组织学形态或免疫组化表达与其他肿瘤有重叠,容易误诊为尿路上皮癌、神经内分泌肿瘤或恶性黑色素瘤,但结合免疫组化可以明确诊断。膀胱副神经节瘤良、恶性判断主要依据为有、无转移。Objective To study the clinicopathologic features,diagnosis and differential diagnosis of paraganglioma of urinary bladder（ PUB）.Methods Twelve cases of paraganglioma of urinary bladder were investigated by clinical findings,histopathology and immunohistochemistry,and related literatures were also reviewed.Results Five of the patients were male and another seven were female.The median age of patients was 50.6 years（ ranged from 34 to 54years）.The tumors in size ranged from 0.8 cm to 4.8 cm in maximum diameter.Microscopic examination revealed that the tumor was composed of the epithelioid cells and spindle-shaped sustentacular cells surrounded by vascularized connective tissue septa.The tumor located at submucosa or the muscular wall with invasive growth or expansive growth.In one case,the tumor cells showed significant nuclear atypia and frequent mitoses.The vascular invasion and necrosis was found.Ki-67 index was about 60%.At 88 months follow-up the patient felt well with no signs of recurrence or metastatisis.Immunohistochemically,the tumor cells were diffusely positive for Cg A,Syn and CD56,but negative for keratin.The spindle-shaped sustentacular cells were positive for S-100.Ten of twelve cases with complete follow-up data had remained free of locally recurrent or metastatic disease through 4 months to 88 months of follow-up.Conclusions Paraganglioma of urinary bladder is a very rare.The histological morphology and immunohistochemical expression of PUB were overlap with other tumors.The differential diagnosis should included urothelial carcinoma,neuroendocrine tumors and malignant melanoma.Immunohistochemical studies can confirm the diagnosis.The main judgment of benign and malignant of paraganglioma of urinary bladder was the presence of metastases.

关 键 词：膀胱肿瘤 副神经节瘤 临床病理特征 鉴别诊断 

分 类 号：R737.14[医药卫生—肿瘤]