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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 investigate the clinical and pathological features, diagnosis and differential diagnosis of paragangliomas (PUB). Methods The clinical and pathological features of 12 cases of paraganglioma of the bladder were studied. Immunohistochemical staining and review of relevant literature were performed. Results In 12 cases, there were 5 males and 7 females, with an average age of onset of 50.6 years (34 to 80 years). The largest diameter of tumor 0.8 ~ 4.8 cm. Microscopic tumor tissue consists of the main cells and supporting cells, which contains a rich blood sinus network formed. Tumor tissue in the bladder submucosa or muscle wall can be manifested as interspersed growth, but also swollen growth and the formation of clear border nodules. One case of atypical tumor cells was obvious, more common mitosis, multiple focal coagulation necrosis, focal tumor thrombus can be seen, the Ki-67 index was 60%, this case was followed up for 88 months without recurrence and metastasis. Immunohistochemistry: tumor cell neuroendocrine markers Cg A, Syn and CD56 diffuse strong (+), epithelial markers (-), supporting cells S-100 (+). 10 cases were followed up data, follow-up time of 4 to 88 months, no recurrence and metastasis. Conclusions Bladder paraganglioma is a rare clinical, histological or immunohistochemical expression that overlaps with other tumors and is easily misdiagnosed as urothelial carcinoma, neuroendocrine tumor or malignant melanoma. However, immunohistochemistry can confirm the diagnosis. Bladder paraganglioma good and malignant judgments mainly based on yes, no transfer.