目的:膀胱副神经节瘤(UBPGLs)是一种罕见的神经内分泌肿瘤,在诊断和手术方面具有挑战性。尚不清楚哪些因素有助于及时的术前诊断。这项研究的目的是确定导致手术前UBPGL诊断缺失的因素。
方法:共有来自中国11个中心的73名患者,纳入来自欧洲6个中心和美国1个中心的51例患者.临床,收集手术和遗传数据,并对手术前和手术后确诊的患者进行比较.使用Logistic回归分析来确定与术前生化检测开始相关的临床因素。
结果:在所有患者中,只有47.6%在手术前确诊。这些患者年轻(34.0vs.54.0年,p<.001),有较大的肿瘤(2.9vs.1.8cm,p<.001),更多的人患有SDHB致病性变异(54.7%vs.11.9%,p<.001)比手术后诊断的那些。术前诊断的患者表现出更多的排尿法术(39.7%vs.15.9%,p=.003),高血压(50.0%vs.31.7%,p=.041)和儿茶酚胺相关症状(37.9%vs.17.5%,p=.012)。多变量逻辑分析表明,年龄较小(<35岁,比值比[OR]=6.47,p=.013),排尿法术(OR=6.79,p=.007),高血压(OR=3.98,p=.011),出汗(OR=41.72,p=0.013)增加了开始术前生化测试的可能性。
结论:大多数UBPGL患者是在手术后诊断的。年轻的年龄,高血压,排尿法术和出汗是协助进行早期生化检测的线索,因此可以及时进行术前诊断。
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.
METHODS: 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.