Childhood Adrenocortical Carcinoma
Adrenocortical carcinoma (ACC) is a cancer of the cortex of the adrenal gland (endocrine-related cancers). There are two types. In one type, the tumor continues to secrete the hormones normally produced by the cortex, including glucocorticoids, mineralocorticoids, and adrenal sex hormones. However, these steroids may be produced in excessive amounts, with negative effects on the body. In the other type, the tumor does not produce these hormones and may go undiscovered until it metastasizes.
Cancer of the adrenal cortex is exceedingly rare: only about 300 cases are diagnosed in the United States each year. Adrenocortical tumors (ACT) represent only about 0.2 % of all malignancies in children. The frequency of ACT is 0.4 per million during the first 4 years of life, 0.1 per million during the subsequent 10 years, and 0.2 per million during the late teens. These tumors also occur in adults, usually during the fourth to fifth decades of life. The...
KeywordsCongenital Adrenal Hyperplasia Adrenocortical Carcinoma Zona Glomerulosa Complete Tumor Resection Zona Fasciculata
- Pinto EM, Chen X, Easton J, Finkelstein D, Liu Z, Pounds S, Rodriguez-Galindo C, Lund TC, Mardis ER, Wilson RK, Boggs K, Yergeau D, Cheng J, Mulder HL, Manne J, Jenkins J, Mastellaro MJ, Figueiredo BC, Dyer MA, Pappo A, Zhang J, Downing JR, Ribeiro RC, Zambetti GP (2015) Genomic landscape of paediatric adrenocortical tumours. Nat Commun 6:6302. doi:10.1038/ncomms7302Google Scholar
- Pinto EM, Morton C, Rodriguez-Galindo C, McGregor L, Davidoff AM, Mercer K, Debelenko LV, Billups C, Ribeiro RC, Zambetti GP (2013) Establishment and characterization of the first pediatric adrenocortical carcinoma xenograft model identifies topotecan as a potential chemotherapeutic agent. Clin Cancer Res 19(7):1740–7Google Scholar
- Ribeiro RC, Pinto EM, Zambetti GP, Rodriguez-Galindo C (2012) The International Pediatric Adrenocortical Tumor Registry initiative: contributions to clinical, biological, and treatment advances in pediatric adrenocortical tumors. Mol Cell Endocrinol 351(1):37–43Google Scholar