The impact of adrenocortical carcinoma hormone secreting status as a predictor of poor survival: a systematic review and meta-analysis

Purpose: 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.

Methods: 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.

Results: 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.

Conclusions: 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.