NOTCH1 and adrenal cortex carcinoma: ACC is highly heterogenous and can be classified into two subtypes.9-11 ACC-I is aggressive, with a median overall survival (mOS) of 3.4 years and enrichment for NOTCH1-activating mutations and solid histology.11,12 ACC-II is more indolent, with a mOS of 23.2 years, TP63 overexpression, predominantly nonsolid (tubular/cribriform) histology, and tendency for lung metastasis.11 ACC-II is more frequent, representing approximately 70% of ACC.