Beckwith-wiedemann syndrome and neural crest tumors. A report of two cases

Pediatr Radiol. 1989;19(4):242-5. doi: 10.1007/BF02386843.


We report 2 cases of thoracic neural crest tumors complicating the course in patients with Beckwith-Wiedemann syndrome (BWS). In the first patient, a thoracic neuroblastoma was fortuitously discovered at age 3 months on a chest film prior to a partial glossectomy. In the follow-up left nephroblastoma and a right kidney simple cyst appeared. In the second patient, a thoracic tumor which proved to be a mature ganglioneuroma was discovered at age 4 years on a follow up spinal radiograph. Although less frequent than nephroblastoma and/or adrenal tumors, the occurrence of thoracic neuroblastoma in BWS suggests that periodic chest radiograph and assays of HVA, VMA and Dopamine should be included in the follow-up protocol.

Publication types

  • Case Reports

MeSH terms

  • Beckwith-Wiedemann Syndrome / complications
  • Beckwith-Wiedemann Syndrome / diagnostic imaging*
  • Child, Preschool
  • Female
  • Ganglioneuroma / complications
  • Ganglioneuroma / diagnostic imaging*
  • Humans
  • Infant, Newborn
  • Male
  • Neural Crest / diagnostic imaging*
  • Neuroblastoma / complications
  • Neuroblastoma / diagnostic imaging*
  • Radiography
  • Thoracic Neoplasms / complications
  • Thoracic Neoplasms / diagnostic imaging*