Recent advances in genetic studies have pointed to further challenges: most ACC tumors contain the fusion myelobastosis viral oncogene homolog-nuclear factor 1B (MYB-NF1B (Persson et al., 2009; Andersson and Stenman, 2016) but also include multiple other cancer-associated gene mutations (Ho et al., 2013; Stephens et al., 2013; Ross et al., 2014; Mitani et al., 2016). The gene discussed is NFIB; the disease is adrenal cortex carcinoma.