Department of Gastroenterological Surgery, Saitama Cancer Center, Saitama, 362-0806, Japan.
Department of Urology, Saitama Cancer Center, Saitama, Japan.
World J Surg Oncol. 2022 Jun 8;20(1):185. doi: 10.1186/s12957-022-02662-7.
Paraganglioma of the urinary bladder (Pub) is rare and presents with clinical symptoms caused by catecholamine production and release. The typical symptoms of Pub are hypertension, macroscopic hematuria, and a hypertensive crisis during micturition. The average size of detected Pubs is approximately 3 cm. Herein, we report a case of a large Pub in which the symptoms were masked by oral medication, precise preoperative diagnosis was difficult, and intraoperative confirmation of tumoral adhesion to the rectum resulted in hypertensive attacks during surgery.
A 64-year-old Japanese male with a history of hypertension and arrhythmia controlled with oral medication presented with a large tumor in the pelvic region, detected on examination for weight loss, with no clinical symptoms. Computed tomography and magnetic resonance imaging revealed a tumor measuring 77 mm in diameter in the posterior wall of the urinary bladder. The border with the rectum was unclear, and the tumor showed heterogeneous enhancement in the solid part with an enhancing hypodense lesion. Cystoscopy revealed compression of the bladder trigone by external masses; however, no tumor was visible in the lumen. Endoscopic ultrasonography-guided fine-needle aspiration revealed CD34-positive spindle-shaped cells in the fibrous tissue, suggestive of a mesenchymal neoplasm. The tumor was suspected to be a gastrointestinal stromal tumor, and surgery was performed. After laparotomy, we suspected that the tumor had invaded the rectum, and total cystectomy and anterior resection of the rectum were performed. Histologically, the tumor cells had granular or clear amphophilic cytoplasm with an oval nucleus and nests of cells delimited by connective tissue and vascular septations. Immunohistochemically, the tumor was positive for chromogranin A, CD56, and synaptophysin, and a diagnosis of paraganglioma of the urinary bladder was confirmed. There was no tumor recurrence at the 7-month follow-up.
This case highlights the importance of careful examination of pelvic tumors, including endocrine testing, for detecting paraganglioma of the urinary bladder in patients with a history of hypertension or arrhythmia.
膀胱副神经节瘤（Pub）较为罕见，其临床表现源于儿茶酚胺的产生和释放。Pub 的典型症状为高血压、肉眼血尿和排尿时发生高血压危象。已检测到的 Pub 平均大小约为 3 厘米。在此，我们报告一例大型 Pub 病例，其症状被口服药物掩盖，术前诊断困难，术中证实肿瘤与直肠粘连，导致手术中发生高血压发作。
一名 64 岁日本男性，因高血压和心律失常接受口服药物治疗，因体检发现盆部有一个大肿瘤就诊，该患者无临床症状，但体重减轻。计算机断层扫描和磁共振成像显示膀胱后侧壁有一个直径为 77 毫米的肿瘤。与直肠的边界不清楚，肿瘤实性部分呈不均匀强化，伴有一个增强的低信号病变。膀胱镜检查显示膀胱三角区受外部肿块压迫；然而，膀胱腔内未见到肿瘤。内镜超声引导下细针穿刺显示纤维组织中有 CD34 阳性梭形细胞，提示间叶性肿瘤。该肿瘤疑为胃肠道间质瘤，因此进行了手术。剖腹手术后，我们怀疑肿瘤侵犯了直肠，因此进行了全膀胱切除术和直肠前切除术。组织学上，肿瘤细胞具有颗粒状或透明嗜酸性细胞质，椭圆形核，细胞巢由结缔组织和血管分隔带界定。免疫组织化学染色显示肿瘤对嗜铬粒蛋白 A、CD56 和突触素呈阳性，确诊为膀胱副神经节瘤。在 7 个月的随访中，没有肿瘤复发。
该病例强调了在有高血压或心律失常病史的患者中，仔细检查盆腔肿瘤（包括内分泌检查）对于发现膀胱副神经节瘤的重要性。
