The occurrence of ovarian cancer in girls may be associated with syndromes such as DICER1 or RTPS (rhabdoid tumor predisposition syndrome); therefore, both germ cell and somatic alterations are also observed in DICER1 (sertoli-Leydig cell tumors) and SMARCA4 (primary small cell carcinoma of the ovary, hypercalcemic type-SCCOHT) (Table 9) [92]. This evidence concerns the gene DICER1 and familial rhabdoid tumor.