The mutation profiles LGACC and head/neck ACC are largely similar though not completely the same.13,14 One shared feature is an accumulation of mutations in the Notch pathway, which include mutant NOTCH1, NOTCH2, and Notch signaling regulator SPEN. This suggests that abnormal Notch signaling may underlie ACC of different sites. The gene discussed is SPEN; the disease is adrenal cortex carcinoma.