Emerging treatment options for adrenocortical carcinoma.

PURPOSE OF REVIEW: Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with limited therapeutic options and poor prognosis. This review highlights recent advances in risk stratification, systemic therapies, surgical approaches and personalized treatment strategies to improve outcomes in ACC.

RECENT FINDINGS: Significant progress has been made in molecular characterization of ACC, enabling more precise risk stratification and prognostication. Novel biomarkers and integrated scoring systems now complement traditional staging methods. Therapeutic advances include refinements in surgical approaches for both localized and metastatic disease, while systemic treatment options are expanding beyond traditional mitotane-based regimens. Immunotherapy and targeted agents show emerging promise, particularly in molecularly selected patients. The role of adjuvant therapy continues to evolve, with recent evidence supporting more selective application based on individual risk assessment. Multimodal strategies combining locoregional and systemic therapies are demonstrating improved outcomes in advanced disease.

SUMMARY: The ACC treatment landscape is undergoing significant transformation, moving toward personalized approaches guided by molecular profiling and risk-adapted strategies. While challenges remain in overcoming treatment resistance and validating new biomarkers, the integration of advanced diagnostics with innovative therapies offers hope for improved patient outcomes. Future progress will depend on collaborative research efforts and the development of robust clinical trials.