Abstract / Summary
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy characterized by marked biological heterogeneity and a high risk of recurrence and progression. While complete surgical resection remains the foundation of treatment for those with localized disease, systemic therapy is commonly used as adjuvant therapy for those with high-risk disease and for those whose ACC is not amenable to curative-intent surgery. Over the past decade, advances in molecular characterization have enhanced the understanding of ACC biology, leading to an increased number of systemic therapies tested for this disease. Unfortunately, the overall activity demonstrated has been low, and the optimal patient selection and treatment sequencing remain poorly defined. This review summarizes the evidence supporting the different systemic therapies currently in use for ACC and strategies for how best to select and sequence these treatment options in patients with advanced disease.