Subsequent studies have shown that germline DICER1 mutations are associated with various malignant and benign tumors, including PPB, cystic nephroma, ovarian sex cord-stromal tumor (OSCST; especially Sertoli-Leydig cell tumor (SLCT)), embryonal rhabdomyosarcoma (ERMS), multinodular goiter, differentiated thyroid carcinoma, nasal chondromesenchymal hamartoma, ciliary body medulloepithelioma, and others (49), (50), (51). This evidence concerns the gene DICER1 and pleuropulmonary blastoma.