It seems likely that biallelic inactivation of both DICER1 allelesis required for the tumor to develop properly, as this phenomenon has been reportedfor various DICER1-related neoplasia, such as PPBs, Wilms tumors, embryonalrhabdomyosarcoma and anaplastic sarcomas of the kidney, and in different subtypes ofthyroid cancer (31, 32, 33, 34, 35, 36, 37). The gene discussed is DICER1; the disease is Nephroblastoma.