Abstract
Adrenocortical carcinoma (ACC) is among the rarest solid malignancies, but is characterized by pronounced biological aggressiveness and a high risk for recurrence. Complete tumor resection (R0) remains the only potentially curative treatment and is the primary determinant of long-term survival. Given the functional heterogeneity of ACC and the frequent presence of hypercortisolism or androgen excess, preoperative evaluation and perioperative management require close interdisciplinary management. Open adrenalectomy continues to be regarded as the gold standard, whereas minimally invasive techniques may be considered in strictly selected cases and within high-volume expert centers. The relevance of systematic lymphadenectomy is increasingly recognized, although its optimal extent remains a matter of debate. Postoperatively, adjuvant therapy is tailored according to individual risk profiles, with mitotane indicated for patients at intermediate or high risk of recurrence. Recurrence is common and should be managed surgically whenever technically feasible. In metastatic disease, multimodal treatment strategies-integrating surgical, interventional radiologic, and systemic approaches-play a central role. Although the overall prognosis remains limited, surgical expertise and treatment within specialized centers are the key for improved patient outcomes.