Endocrine Abstracts

Adrenocortical carcinoma (ACC) is a rare but highly aggressive endocrine tumor with a relatively poor prognosis, which depends on disease stage, resection status, and tumor grading. Surgery remains the cornerstone of treatment and the only realistic chance for cure. However, even after complete resection, a significant proportion of patients remain at high risk for recurrence. Beyond surgery, treatment options are limited, with scientific evidence often based on small retrospective studies, leading to considerable uncertainty in clinical decision-making. This lack of robust data is even more pronounced in pediatric patients, whose management is largely extrapolated from studies in adults. Recent guidelines have provided recommendations for the clinical management of ACC, yet the supporting evidence for many of these recommendations remains weak. From a clinical perspective, some critical questions need to be addressed, such as identifying patients who will benefit most from different adjuvant therapies, as well as refining the management of patients with recurrent disease, where treatment strategies remain uncertain. Additionally, managing patients with advanced ACC presents a significant challenge, as responses to the best available systemic therapy, EDP plus mitotane, remain limited. This presentation will explore the current state of ACC management, highlight ongoing challenges, and discuss potential strategies to improve outcomes.