Wiedemann-Beckwith syndrome (WBS) has attracted a great deal of attention because of its genetic complexity. Individuals with WBS can be identified objectively by anthropometric analysis. Craniofacial anthropometry in conjunction with multivariate statistical analysis can be used to define patterns
Neuroblastoma in a child with Wiedemann-Beckwith syndrome
โ Scribed by Chitayat, David ;Friedman, J. M. ;Dimmick, James E.
- Publisher
- John Wiley and Sons
- Year
- 1990
- Tongue
- English
- Weight
- 445 KB
- Volume
- 35
- Category
- Article
- ISSN
- 0148-7299
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โฆ Synopsis
Abstract
We report on a patient with WiedemannโBeckwith syndrome (WBS) who developed abdominal neuroblastoma. Although WBS patients are known to have a higher incidence of embryonal tumors, this is only the 4th known case of neuroblastoma associated with this syndrome. Chromosomes on peripheral lymphocytes and tumor cells were normal. Children with WBS should be screened for a variety of embryonal neoplasms, not only Wilms tumor.
๐ SIMILAR VOLUMES
## Abstract BeckwithโWiedemann syndrome (BWS) is a genetic disorder associated with an increased risk of childhood tumors. Here we describe a patient with BWS who developed a central nervous system atypical teratoid/rhabdoid tumor (AT/RT). To our knowledge, despite the known cancer predisposition,
Wiedemann-Beckwith syndrome (WBS) is a heterogeneous overgrowth syndrome associated with malformations and an elevated risk of developing embryonal tumors. WBS is a multigenic disorder caused by dysregulation of imprinted growth regulatory genes within the 11p15 region. Elucidation of the genetic ca