The incidental detection of small benign adrenocortical tumors has increased in patients who are investigated with imaging techniques in the upper abdomen. Because these benign lesions are difficult to differentiate from their rare malignant counterparts, the interest to find better tumor markers has increased. In this overview of genetic events in adrenocortical tumors the cancer syndromes Li-Fraumeni, Beckwith-Wiedemann, multiple endocrine neoplasia type 1, congenital adrenal hyperplasia, Carney complex, and McCune-Albright are discussed. Moreover, molecular and cytogenetic analysis on sporadic adrencortical tumors indicate that chromosomes 2, 4, 5, 11, 17, and 18 may harbor genes of importance for the malignant transition of this tumor form. Candidate genes such as IGF2, TP53, and ACT-R are located on these chromosomes and have shown alterations.
*Department of Surgery, Karolinska Hospital, SE-171 76 Stockholm, Sweden. †Department of Molecular Medicine, Karolinska Hospital, Stockhom, Sweden.