Compared with patients with parotid gland ACC, the submandibular gland ACC is more likely to have metastases in the cervical lymph node (21.7% vs. 3.3%) and shows a higher rate of distant metastasis within 1 year after the primary site diagnosis (47.8% vs. 23.3%), a worse overall prognosis, more frequent mutations of MYB/MYBL1 (50% vs. 25%) and abnormal upregulation of the phosphatidylinositol-3 kinase (PI3K) pathway. Here, MYB is linked to adrenal cortex carcinoma.