Current issues in the diagnosis and management of adrenocortical carcinomas

Adrenocortical carcinoma is a relatively rare and heterogenous malignancy with evolving diagnostic and therapeutic approaches. The majority of previous information, mainly derived from small-sized series, considered it as a highly malignant tumor with imminent prognosis. However, recent expanded multicenter and multinational databases have produced a considerable amount of information regarding the relation of tumor extent and prognosis, predictors of biological behavior and response to established and evolving therapeutic modalities. Current management of adrenocortical carcinoma involves a multidisciplinary approach and is based on near-total surgical excision and adjuvant therapy with one or more therapeutic schemes based on tumor biology and staging. Mitotane, with or without chemotherapy, remains the cornerstone of treatment, whereas the role of radiotherapy is evolving. Molecular markers are vigorously explored to stratify patients at high risk for recurrence and exploit newer therapeutic approaches.