Deleterious variants in TP53 are the cause of Li-Fraumeni syndrome (LFS), a cancer predisposition syndrome associated with the development of various tumours: soft tissue sarcoma, osteosarcoma, pre-menopausal breast cancer, brain tumours, adrenocortical carcinoma and leukemias41. The gene discussed is TP53; the disease is adrenal cortex carcinoma.