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. 2024 Jan 8;8(4):bvad170.
doi: 10.1210/jendso/bvad170. eCollection 2024 Feb 19.

Clinical and Pathological Predictors of Death for Adrenocortical Carcinoma

Eduardo Pato  1 , Victor Srougi  1 , Claudia Zerbini  2 , Felipe L Ledesma  2 , Fabio Tanno  1 , Madson Q Almeida  3 , William Nahas  1 , Ana Claudia Latronico  3 , Berenice B Mendonca  3 , Jose L Chambô  1 , Maria Candida B V Fragoso  3
Affiliations

Clinical and Pathological Predictors of Death for Adrenocortical Carcinoma

Eduardo Pato et al. J Endocr Soc. .

Abstract

Adrenocortical carcinoma (ACC) is a rare and lethal disease with a poor prognosis. This study aims to share our 41-year experience as a referral center, focusing on identifying risk factors associated with ACC mortality. Our retrospective analysis included a cohort of 150 adult patients with ACC in all stage categories, treated between 1981 and 2022. Tumor hormonal hypersecretion was observed in 78.6% of the patients, and the median age of diagnosis was 40 years. The majority presented as European Network for the Study of Adrenal Tumors (ENSAT) III or IV (22.9% and 31.2%, respectively), and the overall mortality rate was 54.6%. Independent predictors of death were elevated secretion of cortisol (HR = 2.0), androstenedione (HR = 2.2), estradiol (HR = 2.8), 17-OH progesterone (HR = 2.0), and 11-deoxycortisol (HR = 5.1), higher Weiss (HR = 4.3), modified Weiss (HR = 4.4), and Helsinki scores (HR = 12.0), advanced ENSAT stage (HR = 27.1), larger tumor size (HR = 2.7), higher Ki-67 percentage (HR = 2.3), and incomplete surgical resection (HR = 2.5). Mitosis greater than 5/50 high-power field (HR = 5.6), atypical mitosis (HR = 2.3), confluent necrosis (HR = 15.4), venous invasion (HR = 2.8), and capsular invasion (HR = 2.4) were also identified as independent predictors of death. Knowing the risk factors for ACC's mortality may help determine the best treatment option.

Keywords: adrenocortical carcinoma; hormonal hypersecretion; mortality; risk factors.

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Figures

Figure 1.
Figure 1.
ENSAT stage Kaplan-Meier survival curve: Higher ENSAT stage is associated with mortality in our cohort (P < .001).
Figure 2.
Figure 2.
Weiss score Kaplan-Meier survival curve: Higher Weiss score is associated with mortality in our cohort (P < .001).
Figure 3.
Figure 3.
Modified Weiss score Kaplan-Meier survival curve: Higher modified Weiss score is associated with mortality in our cohort (P < .001).
Figure 4.
Figure 4.
Helsinki score Kaplan-Meier survival curve: Higher Helsinki score is associated with mortality in our cohort (P < .001).
See this image and copyright information in PMC

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