非功能性肌肉浸润性膀胱副神经节瘤--病例报告。

IF 0.7 Q3 MEDICINE, GENERAL & INTERNAL AME Case Reports Pub Date : 2024-09-05 eCollection Date: 2024-01-01 DOI:10.21037/acr-24-50
Joshua S Jue, Jonathan Weinreich, Noel A Armenakas
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引用次数: 0

摘要

背景: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%,不到所有副神经节瘤的 1%。在泌尿生殖道,副神经节瘤最常见于膀胱。这些肿瘤具有侵袭性恶性潜能,因此对局部疾病进行彻底手术切除非常重要。副神经节瘤可能是无功能性的,也可能是有儿茶酚胺分泌的功能性肿瘤。虽然这些肿瘤很罕见,也很难与尿路上皮癌(UC)区分,但术中操作这些肿瘤可能会导致儿茶酚胺激增和术中并发症。术前或术中早期识别这种肿瘤将有助于适当的α受体阻滞,从而将发病率降至最低:在此,我们报告了一例罕见的非功能性副神经节瘤,该肿瘤发生于一名 46 岁男性的膀胱。该病例的显著特点是肿块的位置,需要使用 70 度膀胱镜才能完全观察到膀胱颈附近的情况,而且在切除过程中发现了一个金黄色的无梗肿块。一旦发现该肿块,手术应暂停,以密切监测血流动力学,并评估是否出现高血压危象,然后再继续手术,而无需使用α阻滞剂:结论:疑似局部膀胱副神经节瘤病例应优化血液动力学并进行手术治疗。大体检查发现无柄膀胱肿块,切除时肿瘤呈金黄色,应怀疑为副神经节瘤。应使用血清或尿液中的儿茶酚胺、甲肾上腺素和去甲肾上腺素进行生化评估,以确定肿瘤的功能。
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Non-functional muscle-invasive bladder paraganglioma-a case report.

Background: 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.

Case description: 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.

Conclusions: 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.

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