Why do patients with pheochromocytomas/paragangliomas exhibit significantly inconsistent elevation of catecholamines and their metabolites? a large Chinese cohort study.

IF 3.7 3区医学 Q2 Medicine

Endocrine Pub Date : 2024-11-14 DOI:10.1007/s12020-024-04094-7

Yue Zhou, Yinghan Chen, Wenqian Zhang, Yunying Cui, TianYi Li, Yu Wang, Ming Li, Anli Tong

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引用次数: 0

Abstract

Objectives: Inconsistent elevation of catecholamines (CAs) and metanephrines (MNs) was observed in pheochromocytomas/paragangliomas (PPGLs). This study aimed to explore its potential causes and report the diagnostic efficacy of MNs and CAs in a large Chinese PPGL cohort.

Methods: Clinical data and results of hormone tests were retrospectively collected. The sensitivity of plasma MNs and 24h-urinary CAs was analyzed. Clinical characteristics were compared between patients with positive and negative biochemical results, and among those with inconsistent elevation of MNs and CAs.

Results: Seven hundred and three patients were included, with a median onset age of 41.0 years. Plasma MNs (93% ~ 99%) showed prior diagnostic sensitivity to 24h-urinary CAs (74% ~ 89%). Patients with negative biochemical results presented as a smaller tumor (1.7 cm vs. 4.3 cm, P = 0.001), a higher proportion of normal blood pressure (60.0% vs. 20.5%, P = 0.002) and negative ¹³¹I-meta-iodobenzylguanidine (¹³¹I-MIBG) imaging (55.6% vs. 17.3%, P = 0.012). An earlier age of onset (40.0 vs. 41.0 vs. 44.5 years, P = 0.025), a higher proportion of paroxysmal hypertension (36.5% vs. 29.2% vs. 32.2%, P = 0.005) and the Epinephrine secretion type (79.3% vs. 37.7% vs. 48.0%, P < 0.001) were found in patients with the ratio of MNs/CAs elevation multiples <0.5 than those with the ratio between 0.5 and 2 or >2.

Conclusions: Plasma MNs exhibited a superior sensitivity than 24-h-urinary CAs in the Chinese PPGL diagnosis. Patients with a small tumor, normal blood pressure and negative ¹³¹I-MIBG imaging were prone to express negative biochemical levels. Additionally, an obvious elevation of 24-h-urinary CAs than plasma MNs was correlated to paroxysmal hypertension and the Epinephrine type.

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一项大型中国队列研究：为什么嗜铬细胞瘤/副神经节瘤患者的儿茶酚胺及其代谢物的升高明显不一致？

目的：在嗜铬细胞瘤/副神经节瘤（PPGLs）中观察到儿茶酚胺（CAs）和甲肾上腺素（MNs）的不一致升高。本研究旨在探索其潜在原因，并报告中国大型 PPGL 队列中 MNs 和 CAs 的诊断效果：方法：回顾性收集临床数据和激素检测结果。分析了血浆 MNs 和 24h 尿液 CAs 的敏感性。比较生化结果阳性和阴性患者的临床特征，以及 MNs 和 CAs 升高不一致的患者的临床特征：结果：共纳入 730 名患者，中位发病年龄为 41.0 岁。血浆 MNs（93% ~ 99%）的诊断灵敏度高于 24h 尿液 CAs（74% ~ 89%）。生化结果阴性的患者肿瘤较小（1.7 厘米 vs. 4.3 厘米，P = 0.001），血压正常的比例较高（60.0% vs. 20.5%，P = 0.002），131I-meta-碘苄基胍（131I-MIBG）成像阴性（55.6% vs. 17.3%，P = 0.012）。发病年龄较早（40.0 岁 vs. 41.0 岁 vs. 44.5 岁，P = 0.025）、阵发性高血压比例较高（36.5% vs. 29.2% vs. 32.2%，P = 0.005）和肾上腺素分泌型（79.3% vs. 37.7% vs. 48.0%，P 2.结论：血浆 MNs 在中国 PPGL 诊断中的灵敏度优于 24 小时尿液 CAs。肿瘤较小、血压正常、131I-MIBG 显像阴性的患者容易出现生化水平阴性的情况。此外，24 小时尿液 CAs 明显高于血浆 MNs 与阵发性高血压和肾上腺素型相关。

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来源期刊

Endocrine 医学-内分泌学与代谢

CiteScore

6.40

自引率

5.40%

发文量

期刊介绍： Well-established as a major journal in today’s rapidly advancing experimental and clinical research areas, Endocrine publishes original articles devoted to basic (including molecular, cellular and physiological studies), translational and clinical research in all the different fields of endocrinology and metabolism. Articles will be accepted based on peer-reviews, priority, and editorial decision. Invited reviews, mini-reviews and viewpoints on relevant pathophysiological and clinical topics, as well as Editorials on articles appearing in the Journal, are published. Unsolicited Editorials will be evaluated by the editorial team. Outcomes of scientific meetings, as well as guidelines and position statements, may be submitted. The Journal also considers special feature articles in the field of endocrine genetics and epigenetics, as well as articles devoted to novel methods and techniques in endocrinology. Endocrine covers controversial, clinical endocrine issues. Meta-analyses on endocrine and metabolic topics are also accepted. Descriptions of single clinical cases and/or small patients studies are not published unless of exceptional interest. However, reports of novel imaging studies and endocrine side effects in single patients may be considered. Research letters and letters to the editor related or unrelated to recently published articles can be submitted. Endocrine covers leading topics in endocrinology such as neuroendocrinology, pituitary and hypothalamic peptides, thyroid physiological and clinical aspects, bone and mineral metabolism and osteoporosis, obesity, lipid and energy metabolism and food intake control, insulin, Type 1 and Type 2 diabetes, hormones of male and female reproduction, adrenal diseases pediatric and geriatric endocrinology, endocrine hypertension and endocrine oncology.