Surgical Management of Adrenocortical Carcinoma: An Evidence-Based Approach

Adrenocortical carcinoma frequently presents with sequelae of steroid precursor overproduction and has a proclivity for aggressive local growth, early metastasis, and recurrence. En bloc surgical resection with negative margins is the cornerstone of therapy for localized disease, and re-resection has a role in selected recurrent cases. Presence of occult micrometastatic disease at the time of presentation is confirmed by frequent distant failure after apparent negative margin resection. Data for many aspects of therapy are limited or nonexistent. This review critically considers existing evidence with a particular focus on surgical management.