Wilms' tumor and associated malformations; Report of two cases with WAGR and Drash syndrome

Ozbey H., Boneval C., Kapran Y., Aksoyek S., Salman T., Celik A.

European Journal of Pediatric Surgery, vol.6, no.3, pp.186-188, 1996 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Abstract
  • Volume: 6 Issue: 3
  • Publication Date: 1996
  • Doi Number: 10.1055/s-2008-1066505
  • Journal Name: European Journal of Pediatric Surgery
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.186-188
  • Keywords: Drash syndrome, WAGR syndrome, Wilms' tumor
  • Istanbul University Affiliated: No

Abstract

The combination of Wilms' tumor with aniridia, ambiguous genitalia/gonadoblastoma and mental retardation is defined as WAGR syndrome. The association with pseudohermaphroditism and nephropathy was also described as Drash syndrome. Here we report two cases with WAGR and Drash syndrome. The importance of early recognition and appropriate management of Wilms' tumor with those concurrent malformations is highlighted.