Neoplasms from other organs showing a myogenic differentiation and harboring DICER1 alterations have been reported in the literature (pleuropulmonary blastoma-like peritoneal sarcomas, DICER1 renal sarcomas and rhabdomyosarcomas of the urogenital tract with DICER1 mutations) [67–69], but their relationship (including epigenetic data) has to be elucidated before suggesting a potential unified terminology [70, 71]. Here, DICER1 is linked to pleuropulmonary blastoma.