Therapeutic frontiers in adrenocortical carcinoma: from standards to innovation

Cancer remains the second leading cause of death in America despite cancer-related mortality declining. Adrenocortical carcinoma (ACC) is a rare but aggressive cancer with a poor prognosis that imposes an immense burden on those ill-fated to be touched by this cancer. The 5-year survival rate is below 50% for locally advanced cases, and nearly 75% of patients experience recurrence. Surgical resection is the only curative option, while radiation therapy may be beneficial post-adrenalectomy or in select cases. Mitotane, alone or with etoposide, doxorubicin, and cisplatin, is the primary chemotherapy for advanced or inoperable disease. Immunotherapy is emerging as a promising treatment for advanced ACC due to its proven effectiveness in other cancers. There is still a plethora of new and undiscovered prognostic and pharmaceutical targets for ACC that researchers are investigating. This review explores current and evolving ACC treatments, including surgery, radiotherapy, chemotherapy, and immunotherapy, to aid clinicians in patient management.