Several variants of the germline are characteristic of known predisposing syndromes such as DICER1 (Dicer 1, Ribonuclease III) for pleuropulmonary blastoma (PPB), RB1 for retinoblastoma (RB), TP53 for adrenocortical carcinoma (ACC), and SMARCB1 and SMARCA4 for rhabdoid tumor and small cell ovarian cancer (SCCO), respectively [16]. Here, SMARCA4 is linked to adrenal cortex carcinoma.