Al Subhi Abdul Rahman, Boyle Veronica, Elston Marianne S
Waikato Clinical Campus, University of Auckland, Hamilton 3240, New Zealand.
J Endocr Soc. 2022 Jul 3;6(9):bvac105. doi: 10.1210/jendso/bvac105. eCollection 2022 Sep 1.
Pheochromocytomas and paragangliomas (PPGLs) are known to be rare. However, there is scant literature reporting their epidemiology, particularly whether the diagnosis of PPGL has increased with advances in medical imaging and biochemical and genetic testing.
The primary objective of this systematic review was to determine the annual incidence of PPGLs and change over time.
A systematic review was performed. Medline, Embase, PubMed, and Web of Science Core Collection databases were searched to identify studies reporting PPGL incidence. Studies were eligible for inclusion from the database's inception until August 30, 2021.
A total of 6109 manuscripts were identified; 2282 duplicates were excluded, and a further 3815 papers were excluded after abstract and/or full text review. Twelve studies were included in the final review. The incidence of PPGL ranged from 0.04 to 0.95 cases per 100 000 per year. Incidence increased over time, from approximately 0.2/100,000 individuals in studies performed before 2000, to approximately 0.6/100,000 in studies undertaken after 2010. The mode of diagnosis changed over the same time period, with more patients diagnosed from incidental imaging findings, and fewer at autopsy or from symptoms.
The annual incidence of PPGL has increased over time. Much of this increase is likely from incidental identification of tumors on imaging. However, the epidemiology of PPGL remains understudied, in particular, in associations with altitude, ethnicity, and genetics. To improve early detection and management guidelines, these gaps should be addressed.
嗜铬细胞瘤和副神经节瘤（PPGLs）较为罕见。然而，关于其流行病学的文献报道较少，特别是PPGL的诊断是否随着医学成像、生化及基因检测技术的进步而增加。
本系统评价的主要目的是确定PPGL的年发病率及其随时间的变化。
进行了一项系统评价。检索了Medline、Embase、PubMed和Web of Science核心合集数据库，以识别报告PPGL发病率的研究。从数据库建立至2021年8月30日的研究均符合纳入标准。
共识别出6109篇手稿；排除2282篇重复文献，经摘要和/或全文评审后又排除3815篇论文。最终纳入12项研究进行综述。PPGL的发病率为每年每10万人0.04至0.95例。发病率随时间增加，从2000年前进行的研究中的约0.2/10万，增至2010年后进行的研究中的约0.6/10万。在同一时期，诊断方式发生了变化，更多患者通过偶然的影像学检查发现，而通过尸检或症状诊断的患者减少。
PPGL的年发病率随时间增加。这种增加很大程度上可能是由于影像学检查偶然发现肿瘤。然而，PPGL的流行病学仍研究不足，特别是在与海拔、种族和遗传学的关联方面。为了改进早期检测和管理指南，应填补这些空白。
