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

Clinical and pathological predictors of death for adrenocortical carcinoma

  • Endocrinology, Diabetes and Metabolism

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 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% (HR = 2.3), and incomplete surgical resection (HR = 2.5). Mitosis > 5/50HPF (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.

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