Yuan Yaoji, Su Zhengming, Zhu Rui, Li Xiezhao, Xu Guibin
Department of Urology, The Fifth Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong, People's Republic of China.
Key Laboratory of Enhanced Recovery After Abdominal Surgery, Guangzhou, Guangdong, People's Republic of China.
Int Med Case Rep J. 2021 Nov 12;14:765-771. doi: 10.2147/IMCRJ.S336659. eCollection 2021.
Bladder paraganglioma (BPG) is one of the rare neuroendocrine neoplasms that develops from neural crest cells. It categorizes into functional and non-functional types based on the catecholamines secretion. Currently, functional BPG is predicted in advance based on signs and symptoms of catecholamine excess, such as hypertension and "micturition attacks". However, it is often overlooked because of its rareness. Misdiagnosis of a functional tumor may increase the risk of surgical intervention.
We reported 3 cases of BPG that they were admitted to the hospital due to abdominal pain or gross hematuria. Computed tomography (CT) scans showed space-occupying lesions in the bladders with diameters less than 3cm. There were no typical catecholamine excess symptoms before surgical intervention. Postoperative pathology confirmed BPG after removal of the tumor. We also analyze 69 cases of BPG that has been reported and found that 78.0% cases were functional among the tumors larger than 3cm.
Bladder tumors larger than 3cm in diameter can serve as an additional predictor of functional BPG. Patients who are suspected should undergo magnetic resonance imaging (MRI) scans, 123/131 metaiodobenzylguanidine (MIBG) scan, and have their catecholamine levels tested. Once the diagnosis is confirmed, patients should be started on fluid replacement therapy and adrenergic blockade to abate the disorders associated with catecholamine excess.
膀胱副神经节瘤(BPG)是一种罕见的神经内分泌肿瘤,起源于神经嵴细胞。根据儿茶酚胺分泌情况可分为功能性和非功能性类型。目前,功能性BPG是根据儿茶酚胺过量的体征和症状(如高血压和“排尿发作”)预先预测的。然而,由于其罕见性,它常常被忽视。功能性肿瘤的误诊可能会增加手术干预的风险。
我们报告了3例BPG患者,他们因腹痛或肉眼血尿入院。计算机断层扫描(CT)显示膀胱内有直径小于3cm的占位性病变。手术干预前没有典型的儿茶酚胺过量症状。术后病理在切除肿瘤后确诊为BPG。我们还分析了已报道的69例BPG病例,发现直径大于3cm的肿瘤中78.0%为功能性。
直径大于3cm的膀胱肿瘤可作为功能性BPG的一个额外预测指标。疑似患者应接受磁共振成像(MRI)扫描、123/131间碘苄胍(MIBG)扫描,并检测其儿茶酚胺水平。一旦确诊,应开始对患者进行液体替代治疗和肾上腺素能阻滞剂治疗,以减轻与儿茶酚胺过量相关的紊乱。