RT Book, Section A1 Grubbs, Elizabeth G. A1 Perrier, Nancy D. A1 Evans, Douglas B. A1 Lee, Jeffrey E. A2 Morita, Shane Y. A2 Dackiw, Alan P. B. A2 Zeiger, Martha A. SR Print(0) ID 6163449 T1 Chapter 16. Adrenocortical Carcinoma T2 McGraw-Hill Manual: Endocrine Surgery YR 2010 FD 2010 PB The McGraw-Hill Companies PP New York, NY SN 978-0-07-160645-5 LK accesssurgery.mhmedical.com/content.aspx?aid=6163449 RD 2024/11/09 AB Adrenocortical carcinoma (ACC) is a rare malignant endocrine neoplasm with an estimated incidence of 0.5 to 2 cases per 1 million people annually in the United States,1,2 accounting for 0.02% of all cancers reported annually.1 The prognosis for most patients diagnosed with ACC is disappointingly poor because of delays in diagnosis and the absence of effective systemic therapy. Approximately 50% of affected patients do not survive beyond 2 years after diagnosis, and the 5-year mortality rate approaches 80%.3 ACC has a bimodal age distribution with an increased incidence among children younger than 5 years of age and in individuals in their fourth and fifth decades of life. A slightly higher incidence rate is reported for women than for men.4 Approximately 40% of ACCs produce clinically significant excess amounts of steroid hormones, resulting in characteristic signs and symptoms;5 female patients are more likely to have an associated clinical endocrine syndrome. Surgery remains the only effective curative treatment for ACC. In a 1996 study of risk factors, cigarette smoking and the use of oral contraceptives were found to be associated with the development of ACC.6 An association has also been described between ACC and congenital adrenal hyperplasia.7