For example, the estimated risk of recurrence in patients with triple-negative breast cancer is approximately 43%, and 25% in patients with non-triple-negative breast cancer. Risk of Recurrence of ACC Compared with Other CancersĪdjuvant therapy reduces the risk of recurrence in many cancers, although its use in ACC is still a matter of debate. We also discuss knowledge gaps and provide suggestions for future studies and tools to help treat ACC patients after surgery. In this manuscript, we summarize key data to support the use of adjuvant treatment to improve the clinical outcomes of patients with ACC. Since then, many studies have taken place in an attempt to guide the optimal use of adjuvant therapy for ACC. Adjuvant therapy to reduce the risk of ACC recurrence was reported almost five decades ago. Complete surgical resection is the mainstay of treatment of localized disease, although the recurrence rate can be as high as 70% according to some studies. This difference may be due to improved imaging technology that has led to earlier diagnosis in some patients before becoming symptomatic. This is in contrast to earlier studies, in which most patients presented with stage IV disease. Recent studies indicate that a higher percentage of patients are diagnosed in earlier stages (II–III). Since the first ACC case series was described by Otto Ramsay in 1899, many milestones have been achieved in the disease’s treatment and management, including the introduction of cortisone in the 1940s and mitotane in the 1960s. The role of immunotherapy in metastatic ACC is emerging and if deemed efficacious, then future studies will be needed to ascertain the role of adjuvant immunotherapy in ACC. It is of great importance to establish risk calculators to predict recurrence and to implement molecular profiling of ACC to guide adjuvant therapy. There are multiple knowledge gaps in selecting patients for adjuvant therapy. Retrospective data indicate that radiotherapy is an option for select patients, particularly those with positive resection margins. As mitotane levels can take a few months before reaching therapeutic levels, there is an emerging practice of combining platinum-based chemotherapy with mitotane in the adjuvant setting. However, the use of mitotane is based on retrospective and occasionally conflicting evidence.

Mitotane has been the mainstay adjuvant therapy of ACC. The main predictors of ACC recurrence include advanced disease stage, incomplete surgical resection, cortisol production, certain genetic alterations, and high proliferation rate (Ki-67 proliferation index). Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with high risk of recurrence despite macroscopically complete surgical resection.