PURPOSE Pheochromocytomas and Paragangliomas (PPGL) result in chronic catecholamine excess and serious health complications. A recent study obtained a metabolic signature in plasma from PPGL patients; however, its targeted nature may have generated an incomplete picture and a broader approach could provide additional insights. We aimed to characterize the plasma metabolome of PPGL patients before and after surgery, using an untargeted approach, and to broaden the scope of the investigated metabolic impact of these tumors. DESIGN A cohort of 36 PPGL patients was investigated. Blood plasma samples were collected before and after surgical tumor removal, in association with clinical and tumor characteristics. METHODS Plasma samples were analyzed using untargeted nuclear magnetic resonance (NMR) spectroscopy metabolomics. The data were evaluated using a combination of uni- and multi-variate statistical methods. RESULTS Before surgery, patients with a nonadrenergic tumor could be distinguished from those with an adrenergic tumor based on their metabolic profiles. Tyrosine levels were significantly higher in patients with high compared to those with low BMI. Comparing subgroups of pre-operative samples with their post-operative counterparts, we found a metabolic signature that included ketone bodies, glucose, organic acids, methanol, dimethyl sulfone and amino acids. Three signals with unclear identities were found to be affected. CONCLUSIONS Our study suggests that the pathways of glucose and ketone body homeostasis are affected in PPGL patients. BMI-related metabolite levels were also found to be altered, potentially linking muscle atrophy to PPGL. At baseline, patient metabolomes could be discriminated based on their catecholamine phenotype.

中文翻译：

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嗜铬细胞瘤和副神经节瘤的术前与术后非靶向等离子核磁共振波谱代谢组学。

目的 嗜铬细胞瘤和副神经节瘤 (PPGL) 会导致慢性儿茶酚胺过量和严重的健康并发症。最近的一项研究从 PPGL 患者的血浆中获得了代谢特征；然而，其针对性的性质可能产生了不完整的画面，更广泛的方法可以提供更多的见解。我们的目的是使用非靶向方法来表征 PPGL 患者手术前后的血浆代谢组，并扩大研究这些肿瘤的代谢影响的范围。设计 研究了一组 36 名 PPGL 患者。在手术切除肿瘤之前和之后收集血浆样本，并结合临床和肿瘤特征。方法 使用非靶向核磁共振 (NMR) 光谱代谢组学分析血浆样品。使用单变量和多变量统计方法的组合评估数据。结果 手术前，非肾上腺素能肿瘤患者可根据其代谢特征与肾上腺素能肿瘤患者区分开来。与BMI低的患者相比，高BMI患者的酪氨酸水平显着升高。将术前样本的亚组与其术后样本进行比较，我们发现了包括酮体、葡萄糖、有机酸、甲醇、二甲基砜和氨基酸的代谢特征。发现三个身份不明的信号受到影响。结论 我们的研究表明，PPGL 患者的葡萄糖和酮体稳态途径受到影响。还发现与 BMI 相关的代谢物水平发生了改变，这可能将肌肉萎缩与 PPGL 联系起来。在基线时，