Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, UniversitätsSpital Zürich, Zurich, Switzerland.
Klinik für Endokrinologie, Diabetologie und Klinische Ernährung, UniversitätsSpital Zürich, Zurich, Switzerland; Medizinische Klinik und Poliklinik IV, Klinikum der Universität München, Munich, Germany.
Best Pract Res Clin Endocrinol Metab. 2020 Mar;34(2):101347. doi: 10.1016/j.beem.2019.101347. Epub 2019 Oct 21.
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.
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 与较高的心血管发病率相关，并伴有恶性肿瘤的风险。治疗的首选方法是手术切除。然而，PPGL 存在肿瘤持续存在或复发的终身风险。目前，没有任何临床、生化、组织病理学或影像学特征可以预测或排除恶性行为或肿瘤复发。因此，即使在明显完全手术切除后，也建议进行长期随访。早期发现复发对于减少因儿茶酚胺分泌引起的心血管发病率和死亡率、预防副神经节瘤（PGL）的肿块效应引起的发病率或疾病转移扩散至关重要。由于这些肿瘤的罕见性，目前没有关于长期监测的前瞻性数据。事实上，目前的建议是基于回顾性分析、专家意见和病例研究。本综述的目的是提供关于已知增加复发风险的因素的最新知识概述，这些因素可能影响疾病监测以及生化和影像学随访的可用方法。在此基础上，我们旨在为 PPGL 手术后的患者进行个性化随访提出一种实用的方法。
