Adrenocortical Cancer in the Real World: A Comprehensive Analysis of Clinical Features and Management from the Turkish Oncology Group (TOG)

Introduction Adrenocortical carcinoma (ACC) is a rare yet highly malignant tumor associated with significant morbidity and mortality. This study aims to delineate the clinical features, survival patterns, and treatment modalities of ACC, providing insights into the disease's prognosis. Materials and Methods A retrospective analysis of 157 ACC patients was performed to assess treatment methodologies, demographic patterns, pathological and clinical attributes, and laboratory results. The data were extracted from the hospital's database. Survival analyses were conducted using the Kaplan-Meier method, with univariate and multivariate analyses being performed through the Log-rank test and Cox regression analyses. Results The median age was 45. 89.4% had symptoms at the time of diagnosis. The median tumor size was 12 cm. 117 (79.6%) patients were underwent surgery. A positive surgical border was detected in 26 (24.1%) patients. Adjuvant therapy was administered to 44.4% of patients. The median overall survival for the entire cohort was 44.3 months. Median OS was found to be 87.3 months (95% CI 74.4 ̶ 100.2) in stage 2, 25.8 (95% CI 6.5 ̶ 45.1) in stage 3, and 13.3 (95% CI 7.0 ̶ 19.6) months in stage 4 disease. Cox regression analysis identified age, Ki67 value, Eastern Cooperative Oncology Group performance status (ECOG PS), and hormonal activity as significant factors associated with survival in patients with non-metastatic disease. In metastatic disease, only patients who underwent surgery exhibited significantly improved overall survival (OS) in univariate analyses. Conclusion ACC is an uncommon tumor with a generally poor prognosis. Understanding the defining prognostic factors in both localized and metastatic diseases is vital. This study underscores age, Ki67 value, ECOG PS, and hormonal activity as key prognostic determinants for localized disease, offering critical insights into the complexities of ACC management and potential avenues for targeted therapeutic interventions. Microabstract Adrenocortical cancer is a rare and poor prognostic malignant tumor. The definitions of prognostic factors in localized and metastatic diseases are important. In this paper, we defined the clinical features, management, and prognostic factors related to survival in patients with metastatic and non-metastatic ACC. Cox regression analysis showed that age, Ki67 value, ECOG PS, and hormonal activity were significantly associated with survival in patients with non-metastatic disease. Only patients who underwent surgery had significantly better OS compared with patients without surgery in univariate analyses of metastatic disease.