1. Beckwith-wiedemann syndrome and neural crest tumors. A report of two cases.
- Author
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Sirinelli, D., Silberman, B., Baudon, J., Sinnassamy, P., Gruner, M., Montagne, J., Baudon, J J, and Montagne, J P
- Abstract
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. [ABSTRACT FROM AUTHOR]
- Published
- 1989
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