The Wnt/β-catenin pathway and IGF-2 signaling have been confirmed as altered signaling pathways in ACC patients, while increasing data indicate that the available evidence is inadequate for malignant phenotype and poor prognosis (Berthon et al., 2010; Heaton et al., 2012), especially for the diagnosis of low-grade ACC confined to the adrenal gland (Mete et al., 2018). This evidence concerns the gene IGF2 and adrenal cortex carcinoma.