The Management of Adrenocortical Carcinomas in The Era of Precision Medicine

Adrenocortical carcinomas (ACCs) are rare adrenal tumors that occur in hereditary syndromes or sporadically. Even in sporadic cases, somatic alterations in known susceptibility genes are often detected, emphasizing the growing role of genomic profiling. Recent evidence indicates that ACCs vary in their pathogenetic mechanisms, biochemical phenotype, metastatic potential, and prognosis. While surgery remains the primary treatment for localized ACCs, multiple therapeutic options exist for advanced or metastatic disease, although robust prospective data supporting a genomic profiling-oriented approach are still lacking. To enhance personalized management and improve outcomes in this molecularly complex disease, routine genetic testing for germline mutations along with comprehensive genome profiling for somatic mutations and cluster identification should become standard clinical practice. This review summarizes current evidence on ACC diagnosis and treatment, underscoring the need for a more personalized, cluster-based approach in clinical practice.