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Disease monitoring of patients with pheochromocytoma or paraganglioma by biomarkers and imaging studies.
Best Practice & Research Clinical Endocrinology & Metabolism ( IF 7.4 ) Pub Date : 2019-10-21 , DOI: 10.1016/j.beem.2019.101347
Florentine Schreiner 1 , Felix Beuschlein 2
Affiliation  

Pheochromocytoma and paraganglioma (PPGL) are rare neuroendocrine tumors, a large proportion of which secrete catecholamines. PPGL are associated with a high cardiovascular morbidity and come with a risk of malignancy. The therapy of choice is surgical resection. Nevertheless, PPGL are associated with a lifelong risk of tumor persistence or recurrence. Currently, there are no clinical, biochemical, histopathological or imaging characteristics, which can predict or exclude malignant behavior or tumor recurrence. Therefore, long-term follow-up is recommended even after apparent complete surgical removal. Early detection of recurrence is essential to reduce cardiovascular morbidity and mortality due to catecholamine secretion, to prevent morbidity by mass effects of paraganglioma (PGL) or by metastatic spread of disease.

Due to the rarity of these tumors, no prospective data on long-term surveillance exist. In fact, current recommendations are based on retrospective analyses, expert opinions and case studies. The aim of this review is to provide an overview on the current state of knowledge with regard to known factors that increase the risk of recurrence and might impact disease monitoring as well as the available possibilities for biochemical and imaging follow-up. Based on this overview, we aim to propose a practical approach for a patient-oriented follow-up after surgical removal of a PPGL.



中文翻译:

通过生物标志物和影像学研究监测嗜铬细胞瘤或副神经节瘤患者的疾病。

嗜铬细胞瘤和副神经节瘤(PPGL)是罕见的神经内分泌肿瘤,其中很大一部分分泌儿茶酚胺。PPGL与高心血管疾病的发病率相关,并具有恶性肿瘤的风险。选择的治疗方法是手术切除。然而,PPGL与肿瘤持续或复发的终生风险有关。当前,尚无可预测或排除恶性行为或肿瘤复发的临床,生化,组织病理学或影像学特征。因此,即使在明显的完全手术切除之后,也建议长期随访。早期发现复发对于降低因儿茶酚胺分泌引起的心血管疾病的发病率和死亡率,预防副神经节瘤(PGL)的质量效应或疾病的转移性传播所致的发病率至关重要。

由于这些肿瘤的稀有性,不存在长期监测的前瞻性数据。实际上,当前的建议是基于回顾性分析,专家意见和案例研究的。这篇综述的目的是提供有关已知因素的当前知识概述,这些已知因素会增加复发风险并可能影响疾病监测以及生化和影像学随访的可能可能性。基于此概述,我们旨在为手术切除PPGL之后提出一种针对患者的随访的实用方法。

更新日期:2019-10-21
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