The molecular genetics of adrenocortical carcinoma

Adrenocortical carcinoma (ACC) is a rare endocrine malignancy defined by a heterogeneous clinical presentation, dismal prognosis, and lack of effective therapeutic regimens. The incidence of ACC ranges from 0.5 to 2 cases per million people per year, accounting for 0.02% of all reported cancers [1). Unfortunately, most patients present with metastatic disease which reduces the 5 year survival rate to less than 10% [2]. The resultant limited clinical experience has hampered significant research interest, public awareness and overall support from a limited pool of funding agencies. This review aims to summarize emerging genetic and molecular events implicated in the pathogenesis of ACC that can serve to energize current and future efforts to provide effective diagnostic and therapeutic approaches to this deadly cancer.