It has recently been found that c-kit (CD-117), a tyrosine-kinase receptor involved in growth and development of normal tissues and in some neoplasms [10] expresses in ACC; previous studies have found its expression in 78% of 45 cases[11] and in 78.5% of 14 cases[10], which led some to consider the use of tyrosine kinase inhibitors such as imatinib mesylate as an adjuvant and/or therapeutic tool to manage distant metastases[12]. This evidence concerns the gene KIT and adrenal cortex carcinoma.