肾上腺皮质癌（ACC）因其侵袭性和罕见性而对医疗保健提出了重大挑战。先前的报告表明，与激素分泌肿瘤相关的预后较差。本研究旨在评估 ACC 激素状态对患者肿瘤生存的影响。对 Medline、Embase、Web of Science、CINAHL、CENTRAL 和 ClinicalTrials.gov 数据库进行了全面的文献检索。使用的数据涉及从多变量分析得出的危险比，以尽量减少混杂偏差的影响。随后使用随机效应模型对纳入的研究进行荟萃分析。定量分析中纳入了纳入 4483 名患者的 12 项研究。激素活跃的 ACC 占整个合并患者队列的 48%，并且被发现与显着较差的总生存期相关（HR 1.57，95% 置信区间 1.39-1.78，p<0.001）。无病生存率也受到相应影响（HR 1.32，95% CI 1.11-1.57，p<0.001）。此外，还发现专门分泌 ACC 的皮质醇与死亡或疾病复发风险增加 48% 相关。评估结果之间的研究间统计异质性很小。与不分泌激素的 ACC 相比，产生激素的 ACC 的预后较差，死亡风险增加 57%，复发风险增加 32%。这些发现支持这样的假设：激素的产生意味着不利的肿瘤特异性特征，特别是当导致高皮质醇血症时，表明一种侵袭性疾病表型。© 2024。作者获得 Springer-Verlag GmbH 德国（Springer 旗下公司）的独家许可自然。

Adrenocortical carcinoma (ACC) poses a significant challenge in healthcare due to its aggressive nature and rarity. Prior reports suggest a poorer prognosis associated with hormone-secreting neoplasms. This study aims to assess the impact of ACC hormonal status on patients' oncologic survival.A comprehensive literature search of the Medline, Embase, Web of Science, CINAHL, CENTRAL and clinicaltrials.gov databases was undertaken. Utilized data involved Hazard Ratios derived from multivariable analysis in order to minimize exposure to confounding bias. Included studies were subsequently meta-analyzed using a Random effects model.Twelve studies incorporating 4483 patients were included in the quantitative analysis. Hormonally active ACCs comprised 48% of the entire pooled patient cohort and were found to be associated with significantly worse Overall Survival (HR 1.57, 95% Confidence Interval 1.39-1.78, p < 0.001). Disease-Free Survival was comparably impacted (HR 1.32, 95% CI 1.11-1.57, p < 0.001). Furthermore, cortisol secreting ACCs specifically, were also found to be associated with a 48% increase in the hazard of death or disease recurrence. Interstudy statistical heterogeneity was minimal among evaluated outcomes.Hormone-producing ACCs exhibit a poorer prognosis compared to non-secreting counterparts, with a 57% increased risk of death and a 32% increased risk of recurrence. These findings support the hypothesis that hormone production signifies an adverse tumor-specific feature, particularly when leading to hypercortisolemia, indicating an aggressive disease phenotype.© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.