Fan D G, Wu C L, Huang H J, Wu L, Chen H, Cai S S, Lin N, Lin S Y
Department of Pathology, the Second Affiliated Hospital of Fujian Medical University, Quanzhou 362000, China.
Department of Pathology, Fujian Provincial Hospital, Provincial Clinical Medical College of Fujian Medical University, Fuzhou 350001, China.
Zhonghua Bing Li Xue Za Zhi. 2020 Apr 8;49(4):311-316. doi: 10.3760/cma.j.cn112151-20190928-00535.
To investigate the clinicopathological features, diagnosis, differential diagnosis and immunohistochemical (IHC) characteristics of paraganglioma of urinary bladder (PUB). 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. 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. 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.
探讨膀胱副神经节瘤（PUB）的临床病理特征、诊断、鉴别诊断及免疫组化（IHC）特点。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月期间23例PUB的临床及病理资料。采用EliVision法对细胞角蛋白（CK）、GATA3、CD56、突触素（Syn）、嗜铬粒蛋白A（CgA）、S-100蛋白、HMB45、琥珀酸脱氢酶（SDHB）、八聚体结合转录因子3/4（OCT3/4）和Ki-67进行IHC染色，并复习相关文献。患者中女性14例，男性9例，年龄21～73岁（中位年龄51岁）。临床上，患者表现为头痛、眩晕、心悸、排尿时高血压危象、高血压、视力模糊、肉眼血尿和阵发性苍白。肿瘤大小为0.9～6 cm（平均2.5 cm）。大体上，多数肿瘤呈外生性，在固有层或肌层内界限清楚。肿瘤质地硬，呈结节状，切面呈灰黄色。组织学上，肿瘤生长方式为膨胀性生长，或在固有层或肌层内呈相互穿插的浸润性生长；肿瘤细胞通常呈特征性巢状排列（Zellballen），呈器官样结构；部分病例可见假菊形团。细胞呈圆形或多边形，胞质丰富，嗜酸性或嗜双色性，可含色素颗粒和空泡；细胞核位于中央或偏心，核仁小，偶见一些细胞核呈多形性和深染。部分病例在肿瘤细胞巢周围可见梭形支持细胞。血管丰富，呈裂隙状、血管瘤样或扩张状。免疫组化结果显示，肿瘤细胞GATA3阳性（2/23）、OCT3/4阳性（2/23）、CD56阳性（22/23）、Syn阳性（23/23）、CgA阳性（22/23）、S-100（支持细胞，23/23）和SDHB阳性（23/23）；CK和HMB45阴性；Ki-67指数为1%～5%。随访期间，18例无复发或转移。PUB的诊断依赖于形态学和免疫组化特征，但可能存在组织形态学异质性。最重要的鉴别诊断是浸润性尿路上皮癌。肿瘤细胞可能出现OCT3/4异常胞质表达；该组中SDHB与OCT3/4表达之间无明显相关性。
