CDKN1C and Beckwith-Wiedemann syndrome: Neuroblastoma has been noted in patients with BWS whose underlying mechanism of disease includes loss of CDKN1C gene expression, specifically imprinting control center 2 (IC2) loss of methylation, and paternal uniparental disomy at chromosome 11p15,51 and has also been seen in those with intragenic variants in the maternal copy of CDKN1C. 52This clinical observation may be linked to the high embryonal expression of CDKN1C in the adrenal glands, from which most neuroblastomas arise.53