Diagnosis and management of urinary bladder paragangliomas: A Sino-American-European retrospective observational study

IF 3 3区 医学 Q2 ENDOCRINOLOGY & METABOLISM
Yingxian Pang, Jing Zhang, Jingjing Jiang, Christina Pamporaki, Minghao Li, Nicole Bechmann, Leah Meuter, Yongbao Wei, Haijian Huang, Shenghui Huang, Xunbin Yu, Mercedes Robledo, Miguel J. Soria, Dewen Zhong, Shangyuan Xu, Henri J. L. M. Timmers, Johan F. Langenhuijsen, Xiaofeng Chen, Wanglong Deng, Timo Deutschbein, Hanna Remde, Long Wang, Hanyu Yao, Bin Yan, Annika M. A. Berends, Michiel N. Kerstens, Yazhuo Jiang, Joakim Crona, Ning Xu, Hai Cai, Yanlin Wen, Anguo Wang, Ji Wu, Zongpin Zhang, Jinzhuo Ning, Fan Cheng, Xiang Chen, Jing Wang, Bin Xie, Danlei Chen, Yujun Liu, Longfei Liu, Karel Pacak, Graeme Eisenhofer, Jacques W. M. Lenders
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引用次数: 0

Abstract

Objective

Paragangliomas of the urinary bladder (UBPGLs) are rare neuroendocrine tumours and pose a diagnostic and surgical challenge. It remains unclear what factors contribute to a timely presurgical diagnosis. The purpose of this study is to identify factors contributing to missing the diagnosis of UBPGLs before surgery.

Design, Patients and Measurements

A total of 73 patients from 11 centres in China, and 51 patients from 6 centres in Europe and 1 center in the United States were included. Clinical, surgical and genetic data were collected and compared in patients diagnosed before versus after surgery. Logistic regression analysis was used to identify clinical factors associated with initiation of presurgical biochemical testing.

Results

Among all patients, only 47.6% were diagnosed before surgery. These patients were younger (34.0 vs. 54.0 years, p < .001), had larger tumours (2.9 vs. 1.8 cm, p < .001), and more had a SDHB pathogenic variant (54.7% vs. 11.9%, p < .001) than those diagnosed after surgery. Patients with presurgical diagnosis presented with more micturition spells (39.7% vs. 15.9%, p = .003), hypertension (50.0% vs. 31.7%, p = .041) and catecholamine-related symptoms (37.9% vs. 17.5%, p = .012). Multivariable logistic analysis revealed that presence of younger age (<35 years, odds ratio [OR] = 6.47, p = .013), micturition spells (OR = 6.79, p = .007), hypertension (OR = 3.98, p = .011), and sweating (OR = 41.72, p = .013) increased the probability of initiating presurgical biochemical testing.

Conclusions

Most patients with UBPGL are diagnosed after surgery. Young age, hypertension, micturition spells and sweating are clues in assisting to initiate early biochemical testing and thus may establish a timely presurgical diagnosis.

膀胱副神经节瘤的诊断和治疗:一项中美欧回顾性观察研究
目的膀胱副神经节瘤(UBPGLs)是一种罕见的神经内分泌肿瘤,给诊断和手术带来了挑战。目前仍不清楚哪些因素有助于及时进行术前诊断。本研究旨在确定导致术前漏诊 UBPGLs 的因素。设计、患者和测量纳入了来自中国 11 个中心、欧洲 6 个中心和美国 1 个中心的共 73 名患者以及 51 名患者。收集并比较了手术前和手术后确诊患者的临床、手术和遗传学数据。结果在所有患者中,只有 47.6% 在手术前确诊。与手术后确诊的患者相比,这些患者更年轻(34.0 岁对 54.0 岁,p < .001),肿瘤更大(2.9 厘米对 1.8 厘米,p < .001),有更多 SDHB 致病变体(54.7% 对 11.9%,p < .001)。手术前确诊的患者有更多的排尿痉挛(39.7% 对 15.9%,p = .003)、高血压(50.0% 对 31.7%,p = .041)和儿茶酚胺相关症状(37.9% 对 17.5%,p = .012)。多变量逻辑分析显示,年龄较小(35 岁,比值比 [OR] = 6.47,p = .013)、排尿困难(OR = 6.79,p = .007)、高血压(OR = 3.98,p = .011)和出汗(OR = 41.72,p = .013)会增加启动术前生化检测的概率。年轻、高血压、排尿痉挛和出汗是协助启动早期生化检测的线索,因此可以及时确定手术前诊断。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Clinical Endocrinology
Clinical Endocrinology 医学-内分泌学与代谢
CiteScore
6.40
自引率
3.10%
发文量
192
审稿时长
1 months
期刊介绍: Clinical Endocrinology publishes papers and reviews which focus on the clinical aspects of endocrinology, including the clinical application of molecular endocrinology. It does not publish papers relating directly to diabetes care and clinical management. It features reviews, original papers, commentaries, correspondence and Clinical Questions. Clinical Endocrinology is essential reading not only for those engaged in endocrinological research but also for those involved primarily in clinical practice.
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