Jue Joshua S, Weinreich Jonathan, Armenakas Noel A
Department of Urology, Lenox Hill Hospital, Northwell Health, New York, NY, USA.
Department of Urology, Zucker School of Medicine at Hofstra/Northwell, Hempstead, New York, NY, USA.
AME Case Rep. 2024 Sep 5;8:113. doi: 10.21037/acr-24-50. eCollection 2024.
Extra-adrenal pheochromocytoma (paraganglioma) of the urinary bladder is a rare tumor, accounting for 0.05% of bladder tumors and less than 1% of all paragangliomas. In the genitourinary tract, paragangliomas are most commonly found in the bladder. These tumors have aggressive malignant potential, so complete surgical resection for localized disease is important. Paragangliomas may be non-functional or functional with catecholamine secretions. Although these tumors are rare and difficult to distinguish from urothelial carcinoma (UC), intraoperative manipulation of these tumors may lead to a catecholamine surge and intraoperative complications. Preoperative or early intraoperative recognition of this tumor would facilitate appropriate alpha blockade to minimize morbidity.
Herein we report a rare non-functional paraganglioma arising from the bladder of a 46-year-old male. This case is notable for the location of the mass, requiring a 70-degree cystoscopic lens for complete visualization near the bladder neck, and for the identification of a golden-yellow sessile mass during the resection. Upon visualization of this mass, the operation should be paused for close hemodynamic monitoring and assess for signs of hypertensive crisis prior to continuing without alpha blockade.
Suspected localized bladder paraganglioma cases should be optimized hemodynamically and managed surgically. Visualization of a sessile bladder mass on gross examination with golden-yellow tumor during the resection should prompt suspicion for a paraganglioma. Biochemical evaluation with serum or urine catecholamines, metanephrines, and normetanephrines should be performed to assess for tumor functionality.
膀胱肾上腺外嗜铬细胞瘤（副神经节瘤）是一种罕见肿瘤，占膀胱肿瘤的0.05%，占所有副神经节瘤的不到1%。在泌尿生殖道中，副神经节瘤最常见于膀胱。这些肿瘤具有侵袭性恶性潜能，因此对于局限性疾病进行完整的手术切除很重要。副神经节瘤可能无功能，也可能分泌儿茶酚胺而具有功能。尽管这些肿瘤罕见且难以与尿路上皮癌（UC）区分，但术中对这些肿瘤的操作可能导致儿茶酚胺激增和术中并发症。术前或术中早期识别该肿瘤将有助于进行适当的α受体阻滞以将发病率降至最低。
在此我们报告一例罕见的非功能性副神经节瘤，起源于一名46岁男性的膀胱。该病例的肿块位置值得注意，需要70度膀胱镜镜头才能在膀胱颈附近完全看清，并且在切除过程中发现了一个金黄色的无蒂肿块。看到这个肿块后，手术应暂停，进行密切的血流动力学监测，并在未进行α受体阻滞的情况下继续手术前评估高血压危象的迹象。
疑似局限性膀胱副神经节瘤病例应进行血流动力学优化并进行手术治疗。在切除过程中，大体检查发现膀胱无蒂肿块且肿瘤为金黄色，应怀疑为副神经节瘤。应进行血清或尿液儿茶酚胺、甲氧基肾上腺素和去甲氧基肾上腺素的生化评估，以评估肿瘤的功能。
