Regardless of familial history, the detection rate of disease causing germline TP53 variants has been estimated to be: 50–80% in children presenting with ACC [6–9] or choroid plexus carcinomas [6, 10]; up to 73% in children with rhabdomyosarcoma of embryonal anaplastic subtype [11] and between 3.8 and 7.7% in females with breast carcinoma before 31 years of age [12]. Here, TP53 is linked to adrenal cortex carcinoma.