Nagamani et al. (2009) observed ACC in two out of four patients, all of whom also showed microcephaly, developmental delay, dysmorphic features, and hearing loss. Halgren et al. (2012) described eight patients, harboring interstitial deletions or translocations (one patient) disrupting the ARID1B locus. This evidence concerns the gene ARID1B and adrenal cortex carcinoma.