Individuals affected by germline DICER1 mutations tend to develop multinodular goiter [2], and have an increased risk of developing a variety of tumors, including pleuropulmonary blastoma [2], Wilms tumor (with low frequency) [3], cystic nephroma, embryonal rhabdomyosarcoma, nasal chondromesenchymal hamartoma [4], gynandroblastoma [5], esophageal polyps [6], medulloblastoma [7], and Sertoli-Leydig cell tumors of the ovary [2, 8]. This evidence concerns the gene DICER1 and embryonal rhabdomyosarcoma.