Are we failing in treatment of adrenocortical carcinoma? Lights and shadows of molecular signatures

Adrenocortical carcinoma (ACC) is a rare disease whose prognosis is reported to be invariably poor mainly because of lack of effective and durable treatments, which makes this cancer an orphan disease. Recent studies of functional genomics and, in particular, the results from The Cancer Genome Atlas (TCGA) project have shed new light on the molecular biology of ACC while improving our comprehension of potentially targetable alterations. This review is focused on the shift of methodology in preclinical pharmacology and drug selection for patients with ACC which is rapidly moving from a heuristic approach to a dynamic, deterministic one.