Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) affects women more often than men, with five-year survival rate of about 50% [1]. It is the second most frequent endocrine cancer and the frequency of incidence is twice in females than in males [2, 3]. Approximately half of ACC patients have symptoms of hypersecretion of glucocorticoids and/or androgens while one-third of cases have symptoms related to the tumor mass [1, 4]. The majority of ACC instances are sporadic; however, germline alterations have been linked to 5–10% of ACCs. ACC was seen in several syndromes and develops in about 1% of Li-Fraumeni syndrome patients, usually before the age of 30 years [4–6]. The usage of oral contraceptives, alcohol consumption, and cigarette smoking were found to be linked to the development of ACC [7]. The diagnosis of cancer is based on a thorough evaluation of clinical history and examination for adrenal hormone excess, detailed hormonal workup, and imaging (CT or MRI) before surgery as well as a pathological examination after the tumor has been removed. In the majority of cases, traditional and functional imaging can correctly diagnose an adrenal tumor as ACC. The risk of ACC rises with tumor size, with a higher index of suspicion for larger tumors [8, 9].