Abstract
Adrenocortical carcinoma (ACC) is a rare endocrine malignancy with variable but often aggressive clinical course. Hormonal overproduction can be seen in about half of all ACC cases. When present, the hormonal excess leads to a wide range of metabolic, musculoskeletal, cardiovascular, and infectious complications and adds multiple layers of complexity to the management of ACC. To improve the outcome of patients with hormonally functioning ACC, an effective approach should include parallel efforts to achieve oncologic and hormonal control. An experienced multidisciplinary team is crucial to deliver and coordinate care to manage the specific aspects of this condition. In this review, we summarized the clinical features and management of hormonally functioning ACCs to assist practicing physicians in addressing the complex medical issues that can be seen in the context of this clinical entity.