Vitturi Giacomo, Crisafulli Salvatore, Alessi Ylenia, Frontalini Sara, Stano Marilisa Giustina, Fontana Andrea, Giuffrida Giuseppe, Ferraù Francesco, Trifirò Gianluca, Cannavò Salvatore
Department of Diagnostics and Public Health, University of Verona, Verona, Italy.
Department of Clinical and Community Sciences, University of Milano, Milan, Italy.
J Endocrinol Invest. 2025 Jul 2. doi: 10.1007/s40618-025-02639-9.
The aim of this systematic review and meta-analysis of observational studies is to provide an overview of the global epidemiology of pheochromocytomas and to evaluate the quality of study reporting for the identified studies.
Two bibliographic databases, PubMed and Embase, were searched for studies assessing the epidemiology of pheochromocytoma from inception until December 2024. Original observational studies written in English and reporting pheochromocytoma prevalence and/or incidence for a well-defined geographic area were included. Two reviewers independently screened the retrieved articles for inclusion, extracted relevant data, and carried out the evaluation of the quality of study reporting. A narrative synthesis of the results was provided, along with a meta-analysis of both prevalence and incidence of pheochromocytoma.
A total of 14 studies were included in the systematic review, of which 13 were included in the meta-analysis. The pooled prevalence of pheochromocytoma was 19.8 (95% CI: 9.6-40.8) cases per 1,000,000 individuals, and the incidence rate was 1.9 (95% CI: 1.2-2.6) cases per million person-years. Substantial between-study heterogeneity was observed for both prevalence and incidence rates (I = 98.7 for both), mainly due to different geographic areas and the use of different data sources. The quality of study reporting was rated as high for only one study, low for four studies, and medium for the remaining nine.
Overall, our systematic review and meta-analysis of observational studies revealed significant between-study heterogeneity of prevalence of pheochromocytoma amounting to around 20 per million individuals. Establishing national registries using standardized diagnostic and clinical parameters, along with well-defined selection and analysis criteria, may provide better insight into the epidemiology and the course of this rare disease.
本观察性研究的系统评价和荟萃分析旨在概述全球嗜铬细胞瘤的流行病学情况，并评估所纳入研究的报告质量。
检索了两个文献数据库，即PubMed和Embase，以查找从开始到2024年12月评估嗜铬细胞瘤流行病学的研究。纳入以英文撰写的、报告明确地理区域嗜铬细胞瘤患病率和/或发病率的原始观察性研究。两名审阅者独立筛选检索到的文章以确定是否纳入，提取相关数据，并对研究报告质量进行评估。提供了结果的叙述性综合分析，以及嗜铬细胞瘤患病率和发病率的荟萃分析。
系统评价共纳入14项研究，其中13项纳入荟萃分析。嗜铬细胞瘤的合并患病率为每100万人中有19.8例（95%置信区间：9.6 - 40.8），发病率为每百万人口年1.9例（95%置信区间：1.2 - 2.6）。在患病率和发病率方面均观察到研究间存在显著异质性（两者I² = 98.7），主要是由于地理区域不同和数据源使用不同。只有一项研究的报告质量被评为高，四项研究为低，其余九项为中等。
总体而言，我们对观察性研究的系统评价和荟萃分析显示，嗜铬细胞瘤患病率在研究间存在显著异质性，约为每百万个体20例左右。使用标准化诊断和临床参数以及明确的选择和分析标准建立国家登记册，可能有助于更好地了解这种罕见疾病的流行病学情况及其病程。
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