The study by Bielamowicz et al. presents two cases of pediatric CNS embryonal tumors initially diagnosed with EWSR1-PLAGL1 rearrangements, which at relapse were reclassified as INI-1 deficient tumors due to the acquisition of SMARCB1 alterations and loss of INI-1 expression [64]. This evidence concerns the gene SMARCB1 and embryonal neoplasm.