嗜铬细胞瘤/副神经节瘤病史患者血糖调节异常的长期持续性

Long-Term Persistence of Glycemic Dysregulation in Patients With a History of Pheochromocytoma/Paraganglioma.

作者信息

Fischer Alessa, Remde Hanna, Pamporaki Christina, Dischinger Ulrich, Bechmann Nicole, Robledo Mercedes, Wang Katharina, Vetter Diana, Oberholzer José, Morand Grégoire B, Mueller Simon Andreas, Huber Alexander, Fritsch Ralph, Gruber Sven, Hantel Constanze, Zitzmann Kathrin, Reincke Martin, Auernhammer Christoph J, Pacak Karel, Grossman Ashley B, Beuschlein Felix, Nölting Svenja

机构信息

Department of Endocrinology, Diabetology and Clinical Nutrition, University Hospital Zurich (USZ), University of Zurich (UZH), Zurich 8091, Switzerland.

Department of Internal Medicine I, Division of Endocrinology and Diabetes, University Hospital, University of Würzburg, Würzburg 97080, Germany.

出版信息

J Clin Endocrinol Metab. 2025 Aug 7;110(9):e2966-e2976. doi: 10.1210/clinem/dgae901.

DOI:10.1210/clinem/dgae901

PMID:39745823

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12342352/

Abstract

CONTEXT

Pheochromocytomas and paragangliomas (PPGLs) are rare endocrine tumors that frequently produce catecholamines. Catecholamine-induced cardiometabolic complications substantially contribute to increased morbidity and mortality in PPGL patients prior to surgical resection.

OBJECTIVE

To determine whether markers of elevated cardiometabolic risk persist in patients following PPGL resection.

METHODS

In this retrospective analysis of a multicenter cohort of patients with PPGLs participating in the prospective ProsPheo study and the ENS@T registry, cardiometabolic risk factors, including glycemic status, dyslipidemia, and body mass index (BMI), were assessed in patients with PPGL at diagnosis and during follow-up. Patients with a history of resected PPGL were compared to a control group with nonfunctioning adrenal adenomas from the ENS@T registry.

RESULTS

Patients with a present PPGL or a history of PPGL (n = 188), a metastatic PPGL (n = 27), or a known susceptibility gene pathogenic variant (PV) for the development of PPGL without a history of PPGL (n = 44) were included. We compared the asymptomatic PV carriers to patients with a history of PPGL: those with a history of PPGL showed a significantly higher prevalence of hyperglycemic disorders (P = .013) compared with asymptomatic PV carriers. In patients with a history of PPGL and at least 12 months of follow-up post surgery (n = 113), the prevalence of hyperglycemic disorders (P < .001), as well as the mean HbA1c (5.63%, SD 0.43%), were significantly higher, compared to a control group with nonfunctioning adrenal adenomas (n = 76) of similar age and BMI (HbA1c 5.45%, SD 0.40%; P = .004).

CONCLUSION

Glycemic disturbances persist long-term after the resection of PPGL.

摘要

背景

嗜铬细胞瘤和副神经节瘤（PPGLs）是罕见的内分泌肿瘤，常分泌儿茶酚胺。儿茶酚胺诱导的心脏代谢并发症在很大程度上导致了PPGL患者手术切除前发病率和死亡率的增加。

目的

确定PPGL切除术后患者心脏代谢风险升高的标志物是否持续存在。

方法

在这项对参与前瞻性ProsPheo研究和ENS@T注册研究的多中心PPGL患者队列的回顾性分析中，在诊断时和随访期间对PPGL患者的心脏代谢风险因素进行了评估，包括血糖状态、血脂异常和体重指数（BMI）。将有PPGL切除史的患者与ENS@T注册研究中患有无功能肾上腺腺瘤的对照组进行比较。

结果

纳入了现患PPGL或有PPGL病史的患者（n = 188）、转移性PPGL患者（n = 27）或有已知PPGL发生易感性基因致病变异（PV）但无PPGL病史的患者（n = 44）。我们将无症状PV携带者与有PPGL病史的患者进行了比较：有PPGL病史的患者与无症状PV携带者相比，高血糖疾病的患病率显著更高（P = 0.013）。在有PPGL病史且术后至少随访12个月的患者（n = 113）中，与年龄和BMI相似的无功能肾上腺腺瘤对照组（n = 76）相比，高血糖疾病的患病率（P < 0.001）以及平均糖化血红蛋白（HbA1c）（5.63%，标准差0.43%）显著更高（HbA1c 5.45%，标准差0.40%；P = 0.004）。