Simultaneous occurrence of right adrenocortical tumor and left adrenal neuroblastoma in an infant with Beckwith-Wiedemann syndrome

Pediatr Blood Cancer. 2008 Nov;51(5):695-8. doi: 10.1002/pbc.21694.


Children with Beckwith-Wiedemann syndrome (BWS) have increased risk for development of embryonal tumors. We present the case of an infant with BWS who has hypomethylation of LIT1 gene in the 11p15.5 chromosomal region and at 6 months of age presented with simultaneous occurrence of neuroblastoma arising from the left adrenal gland and a right adrenocortical tumor. She underwent surgical resection of both tumors and remains tumor free 18 months after surgery.

Publication types

  • Case Reports

MeSH terms

  • Adrenal Cortex Neoplasms / complications*
  • Adrenal Gland Neoplasms / complications*
  • Beckwith-Wiedemann Syndrome / complications*
  • Beckwith-Wiedemann Syndrome / genetics
  • DNA Methylation
  • Female
  • Humans
  • Infant
  • Neoplasms, Multiple Primary / complications*
  • Neuroblastoma / complications*
  • Potassium Channels, Voltage-Gated / genetics


  • KCNQ1OT1 long non-coding RNA, human
  • Potassium Channels, Voltage-Gated