Adrenocortical Carcinoma and Adrenal Metastasis

Adrenocortical carcinoma and adrenal metastases are rare. A thorough history and physical are integral for evaluation. Routine genetic testing should be considered especially in patients with a significant family history. Patients may present with symptoms related to the hormones being overproduced if the tumor is functional, with compressive effects of the mass if the tumors are nonfunctional, or with no symptoms in the setting of an incidentaloma. Laboratory evaluation focuses on determination of whether hormones are being produced by the tumors. Labs evaluated include plasma or 24-h urine metanephrine and normetanephrine levels, ACTH, aldosterone, renin, DHEA-S, 11-deoxycortisol, testosterone, estradiol, and 17-OH progesterone. 1 mg dexamethasone suppression test is performed in those without overt signs of Cushing syndrome, and 24-h urine for cortisol in those with overt signs of Cushing syndrome. An adrenal protocol CT is the preferred initial imaging study. Characteristics with increasing risk for malignancy include size >4 cm, Hounsfield units >10, washout % < 50–60%, heterogeneous appearance, irregular borders, calcifications or necrosis, vascular thrombus, direct invasion of adjacent structures, and/or presence of lymphadenopathy. Biopsy is rarely indicated. Surgery is the only treatment modality alone that can achieve cure, and open transabdominal adrenalectomy remains the approach of choice.