Diagnosis and Management of Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare, aggressive malignancy with symptoms arising from mass effect or hormonal excess, particularly hypercortisolism. Accurate diagnosis requires comprehensive clinical, hormonal, radiologic, and histopathologic evaluation. Surgical resection is the only curative treatment. Treatment decisions are guided by risk stratification based on stage, Ki67 index, and resection margin status. Mitotane, the only Food and Drug Administration-approved drug for ACC, is combined with etoposide, doxorubicin, and cisplatin as the standard treatment for patients with recurrent disease. Investigational therapies include tyrosine kinase inhibitors, immune checkpoint inhibitors, image-guided locoregional therapies, radiation therapy, and cell-based therapies.