膀胱嗜铬细胞瘤14例临床病理分析

A clinicopathological study of 14 cases of bladder pheochromocytoma

目的:探讨膀胱嗜铬细胞瘤的临床病理学特征、病理诊断以及鉴别诊断。方法:搜集14例膀胱嗜铬细胞瘤患者的临床病理资料,回顾性总结膀胱嗜铬细胞瘤的临床及病理学特征,复习相关文献。结果:14例患者中男性8例、女性6例;年龄14-63岁,平均年龄46.7岁。肿瘤最大径从0.2-8 cm不等,平均最大径2.5 cm。以排尿后头痛、心悸、血压升高为临床主要症状。镜下可见较一致的多边形或圆形上皮样肿瘤细胞,排列成条索、巢团及片状结构,形成典型的zellballen细胞巢,无包膜,在膀胱壁中浸润性生长;肿瘤间质富含薄壁血管,大部分血管呈血窦状、无显著扩张;大部分细胞较一致,散在少数胞体形状不规则、核大深染的瘤细胞,部分胞浆略嗜碱性,核分裂罕见,瘤巢周边见散在梭形细胞。免疫组织化学显示肿瘤细胞CgA、Syn阳性,增殖指数Ki-67阳性1%-10%不等,瘤巢周边梭形细胞S-100阳性;瘤细胞AE1/AE3阴性。9例采用经尿道膀胱肿物切除,其中4例复发。5例采用膀胱部分切除术,其中2例复发。结论:膀胱嗜铬细胞瘤是发生于膀胱的罕见肿瘤,需要依赖组织形态与免疫组织化学特点与其它膀胱肿瘤进行鉴别诊断;单纯肿瘤切除容易局部复发,引起转移的恶性病例少见。

Objective： To explore the clinicopathologic characteristics and histopathological diagnosis of bladder pheochromocytoma. Methods： A total of 14 cases of bladder pheochromocytoma were retrospectively retrieved from the surgical pathology files of Peking Union Medical College Hospital. The clinical and pathological findings were analyzed with a review of the literature. Results： Of the 14 patients with bladder pheochromocytoma, eight were males and six were females. ~ae median age was 46.7 years （ranged from 14 to 63）, and the median maximum diameter of the tumors was 2.5 cm （ranged from 0.2 to 8）. Microscopic examination showed that the tumor cells were polygonal to spindle-shaped arranged into trabecular, solid or zellballen pattern in a rich vascular network. The tumor was typically not well circumscribed with infiltration of bladder wall. Immunohistochemical staining showed that CgA and Syn were positive for tumor cells, and S-100 was positive for the spindle cells around tumor nests. AE1/AE3 was typically negative. Ki-67 index ranged from 1% to 10%. Nine cases received transurethral resection of bladder tumor, 4 cases of which had local recurrence. Five cases used bladder resection, 2 cases of which had local recurrence. Conclusion： Pheochromocytoma is a rare tumor of the bladder, and the pathological diagnosis is based on morphological and immunohistochemical characteristics. Local recurrence following tumor resection is common, while metastasis is rare.