Adrenocortical carcinoma (ACC) is a rare cancer that has two peaks in incidence: between birth and 4 years, and after the 5th decade of life. Inheriting a mutation in the tumour suppressor gene, TP53, increases the risk of developing ACC. In Southern Brazil, mutation at a particular site in TP53 - R337H - is more common than in other populations and is responsible for a higher occurrence of ACC in this region. However, even amongst people carrying the TP53 R337H mutation, there is large variability in cancer risk and the age of cancer onset, indicating that other factors may influence cancer development.
To understand more about ACC risk, Mirvat Surakhy, Marsha Wallace and colleagues from Gareth Bond’s laboratory studied the genetics of childhood and adult ACC cohorts in Southern Brazil. Their work, published in the British Journal of Cancer, identifies a genetic alteration in the alcohol dehydrogenase gene, ADH7, that significantly associates with differences in the age of ACC onset. Since ADH7 is a component of the druggable retinoic acid pathway, this work has implications for developing new treatments for ACC.