Systemic therapy for adrenocortical carcinoma: a review

Abstract

Adrenocortical carcinoma (ACC) is a rare and aggressive disease with an incidence of 1 per million people. Diagnosis occurs due to compressive symptoms from a large tumor, hormonal production, or incidental discovery on imaging studies. Surgery has been the mainstay of treatment and offers the best chance for a cure. However, local recurrence and metastatic disease are common. Survival at 5 years ranges from 82% for stage I disease to 13% for stage IV disease defined by distant metastases. Seventy percent of patients present with stage III or IV disease. Therefore, many patients require systemic therapy. Adjuvant treatment with mitotane, the only approved medication for ACC, has been used since 1960. Adjuvant radiation to the tumor bed is suggested for patients with a high risk for recurrence. Once the disease becomes metastatic, the current treatment is palliative chemotherapy with etoposide, doxorubicin, and cisplatin (EDP) with mitotane. The EDP + mitotane regimen has a response rate of 23%. Targeted systemic therapies have been less successful than this regimen. Ablative techniques are often used for metastatic disease. More therapies continue to be explored and collaborative groups have formed around the world to work toward improved options for this patient population.