A total of seven somatic variants, two variants in TP53 (c.375G>A:p.T125 = and c.749C>T:p.P250L), three variants in CTNNB1 (c.110C>G:p.S37C, c.121A>G:p.T41A and c.133T>C:p.S45P), one variant in PRKAR1A (c.545C>A:p.T182K) and one variant in ZNRF3 (c.433C>T:p.R145X), were identified in five of the seven ACC cases (71.4%, Fig 1). Here, ZNRF3 is linked to adrenal cortex carcinoma.