adrenocortical carcinoma (acc) is a rare, highly aggressive cancer of adrenal cortical cells, which may occur in children or adults. accs may be "functional", producing steroid hormones and consequent endocrine dysfunction similar to that seen in many adrenocortical adenomas, but many are not. due to their location deep in the retroperitoneum, most adrenocortical carcinomas are not diagnosed until they have grown quite large. they frequently invade large vessels, such as the renal vein and inferior vena cava, as well as metastasizing via the lymphatics and through the blood to the lungs and other organs. the most effective treatment is surgery, although this is not feasible for many patients, and the overall prognosis of the disease is poor. chemotherapy, radiation therapy, and hormonal therapy may also be employed in the treatment of this disease.