Objective: To investigate the clinicopathological features, diagnosis, differential diagnosis and immunohistochemical (IHC) characteristics of paraganglioma of urinary bladder (PUB). Methods: The clinical and pathological data of 23 cases of PUB were collected at the Second Affiliated Hospital of Fujian Medical University (7 cases); Fujian Provincial Hospital (8 cases); Fujian Medical University Union Hospital (6 cases); and First Affiliated Hospital of Fujian Medical University (2 cases) from May 2010 to November 2018. IHC staining for CK, GATA3, CD56, Syn, CgA, S-100 protein, HMB45, SDHB, OCT3/4 and Ki-67 was done using EliVision method; and the relevant literature was reviewed. Results: There were 14 women and 9 men, aged ranged from 21 to 73 years (median 51 years). Clinically, patients presented with headache, vertigo, palpitation, hypertensive crisis during micturition, hypertension, blurred vision, gross hematuria and paroxysmal pallor. The tumor sizes ranged from 0.9 to 6 cm (mean2.5 cm). Macroscopically, most tumors were exophytic and well delineated within the lamina propria or muscularis propria. The tumors were firm and nodular and showed grayish-tan cut surface. Histologically,the tumor growth pattern was expansive or showed interpenetrating infiltrative growth within the lamina propria or muscularis propria; the tumor cells were typically arranged in distinctive nests (Zellballen) with organoid arrangement; pseudo-rosette were seen in some cases. The cells were rounded or polygonal and had rich, acidophilic or amphophilic cytoplasm and may contain pigmented granules and vacuoles; the nuclei were central or eccentric, with small nucleoli, although occasionally some nuclei were pleomorphic and hyperchromatic. Spindled sustentacular cells could be seen around the nests of tumor cells in some cases. There were abundant vessels that were fissure-like, hemangioma-like or dilated. By IHC, the tumor cells were positive for GATA3 (2/23), OCT3/4 (2/23), CD56 (22/23), Syn (23/23), CgA (22/23), S-100 (sustentacular cell, 23/23) and SDHB (23/23); and negative for CK and HMB45; Ki-67 index was 1%-5%. At follow-up, there was no recurrence or metastasis in 18 cases. Conclusions: The diagnosis of PUB relies on the morphologic and IHC features; but there may be histomorphologic heterogeneity. The most important differential diagnosis is invasive urothelial carcinoma. The tumor cells may show aberrant cytoplasmic expression of OCT3/4; there is no clear correlation between SDHB and OCT3/4 expression in the group.
目的： 探讨膀胱副神经节瘤（PUB）的临床病理特点、诊断、鉴别诊断及免疫特征。 方法： 收集福建医科大学附属第二医院（7例）、福建省立医院（8例）、福建医科大学附属协和医院（6例）及福建医科大学附属第一医院（2例）2010年5月至2018年11月手术切除的23例PUB临床及病理资料，采用免疫组织化学EliVision法检测细胞角蛋白（CK）、GATA3、CD56、突触素、嗜铬粒素A（CgA）、S-100蛋白、HMB45、SDHB、OCT3/4及Ki-67，并复习文献综合分析。 结果： 患者中女性14例，男性9例，年龄21~73岁（平均年龄51岁），临床上出现反复头痛、头晕及心悸，排尿性晕厥，血压升高，视力模糊，肉眼血尿，阵发性面色苍白等症状。肿物最大径0.9~6.0 cm（平均最大径约2.5 cm）。大体观察：大部分肿物凸向腔内，边界尚清，位于黏膜固有层或固有肌层，切面呈结节样，灰黄、灰褐色，质地中等。镜下观察：低倍镜下肿瘤细胞在黏膜固有层及固有肌层间膨胀性生长或穿插浸润性生长，肿瘤细胞呈巢状（Zellballen）、器官样排列，少部分病例肿瘤细胞排列成菊形团样结构；细胞较大，胞质丰富、嗜酸性或嫌色性，少数病例胞质内或间质可见色素颗粒及空泡，细胞核圆形、卵圆形，部分呈短梭形，居中并可见奇异型核及瘤巨细胞，部分细胞可见小核仁，少数病例细胞巢周边可见梭形支持细胞，间质血管丰富，呈裂隙样、血管瘤样或扩张，核分裂象罕见。免疫表型：GATA3（2/23）、OCT3/4（2/23）、CD56（22/23）、突触素（23/23）、CgA（22/23）、S-100蛋白（支持细胞，23/23）、SDHB（23/23）阳性表达；CK、HMB45均阴性；Ki-67阳性指数1%~5%。随访18例均未见复发及转移。 结论： PUB诊断依赖于形态学及免疫组织化学染色，组织形态学可见异质性，最主要与浸润性尿路上皮癌进行鉴别诊断。未发现SDHB表达与OCT3/4表达有某些明确相关性，肿瘤细胞可异常胞质表达OCT3/4。.
Keywords: Diagnosis, differential; OCT3/4; Paraganglioma; Succinate dehydrogenase; Urinary bladder neoplasms; Zinc fingers.