Like PLAGL1- and PLAGL2-amplified tumors, PLAG1-fused tumors show high expression of typical PLAG family downstream genes, dysregulation of imprinted genes, and overexpression of the candidate drug targets RET and CYP2W1. We therefore propose to rename the previous CNS embryonal tumor with PLAGL amplification (ET, PLAGL), which only includes PLAGL1/2-amplified tumors, to CNS embryonal tumor with PLAG family gene alteration (ET, PLAG) with a further specification of the three subtypes PLAGL1-amplified, PLAGL2-amplified, and PLAG1-fused. Here, PLAGL2 is linked to central nervous system embryonal neoplasm.