Beckwith-wiedemann syndrome and neural crest tumors. A report of two cases.
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.
Sirinelli
D
D
Service de Radiologie Pédiatrique, Hôpital Trousseau, Paris, France.
Silberman
B
B
Baudon
J J
JJ
Sinnassamy
P
P
Gruner
M
M
Montagne
J P
JP
D001506:Beckwith-Wiedemann Syndrome / Q000150:complications / Q000000981:diagnostic imaging*; D002675:Child, Preschool; D005260:Female; D005729:Ganglioneuroma / Q000150:complications / Q000000981:diagnostic imaging*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009432:Neural Crest / Q000000981:diagnostic imaging*; D009447:Neuroblastoma / Q000150:complications / Q000000981:diagnostic imaging*; D011859:Radiography; D013899:Thoracic Neoplasms / Q000150:complications / Q000000981:diagnostic imaging*