In addition to PPB, DICER1 syndrome includes, among others, Wilms’ tumor, cystic nephroma, clear cell sarcoma, ovarian Sertoli-Leydig cell and granulosa cell tumors, testicular Sertoli-Leydig cell tumors, medulloblastoma, neuroblastoma, seminoma germ cell tumors, pituitary blastoma, pineoblastoma, ciliary body medulloepithelioma, thyroid nodular hyperplasia, papillary and follicular thyroid carcinomas, rhabdomyosarcoma, fibrosarcoma, Ewing sarcoma, osteosarcoma, hepatoblastoma and hepatocellular carcinoma cancer and pineoblastoma [11]. This evidence concerns the gene DICER1 and pleuropulmonary blastoma.