ZCCHC7 is a zinc finger CCHC-type protein functioning as a component of the TRAMP-like polyadenylation complex, with primary roles in nuclear RNA quality control and ribosomal RNA processing. As a putative homolog of yeast Air2p, ZCCHC7 localizes to the nucleolus where it participates in the Trf4/5-Air1/2-Mtr4 polyadenylation-dependent surveillance pathway 1. The protein facilitates degradation of aberrant noncoding RNAs through interaction with the nuclear exosome machinery 2. ZCCHC7 binds RNA and associates with nuclear polyadenylation-dependent rRNA and snRNA catabolic processes. Disease relevance spans both hematologic malignancies and solid tumors. In B-cell acute lymphoblastic leukemia (B-ALL), PAX5::ZCCHC7 rearrangements occur in approximately 1-2.5% of cases, typically as additional genetic abnormalities co-occurring with other subtype-defining rearrangements 3. In diffuse large B-cell lymphoma (DLBCL), ZCCHC7 expression associates with patient survival outcomes as part of an RNA-binding protein prognostic signature 4. During B-cell lymphoma transformation, enhancer retargeting mediated ZCCHC7 upregulation causes nucleolar dysregulation and altered 5.8S rRNA processing, rewiring protein synthesis for oncogenic advantage 1. ZCCHC7 also participates in enhancer release and retargeting mechanisms activating disease-susceptibility loci 5. These findings establish ZCCHC7 as a functionally relevant gene in hematologic malignancy progression with potential therapeutic targeting implications.