Although mutations in RB1 are often considered necessary for retinoblastoma initiation, the transition into retinoblastoma may require further changes in key genes, such as KIF14, E2F3, MYCN, DDX1, MDM4, OTX2, DEK, CDH1, BCOR and RBL2 [2]. This evidence concerns the gene OTX2 and retinoblastoma.