Most cases are sporadic, though ACCs have been described in at least eight hereditary cancer syndromes, including Li-Fraumeni syndrome (TP53 gene), Beckwith-Wiedemann syndrome (abnormalities in 11p15), MEN1 (MEN1 gene), familial adenomatous polyposis (FAP gene), neurofibromatosis type 1 (NF1 gene), and CNC (PRKAR1A gene) [58, 59]. Here, MEN1 is linked to Beckwith-Wiedemann syndrome.