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Eur J Pediatr Surg 1996; 6(3): 186-188
DOI: 10.1055/s-2008-1066505
Case report

© Georg Thieme Verlag KG Stuttgart · New York

Wilms' Tumor and Associated Malformations; Report of Two Cases with WAGR and Drash Syndrome

H.  Özbey1 , C.  Boneval1 , Y.  Kapran2 , S.  Aksöyek1 , T.  Salman1 , A.  Çelik1
  • 1Department of Pediatric Surgery, University of Istanbul, Istanbul Faculty of Medicine, Istanbul, Turkey
  • 2Department of Pathology, University of Istanbul, Istanbul Faculty of Medicine, Istanbul, Turkey
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Publication History

Publication Date:
25 March 2008 (online)

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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.

Zusammenfassung

Die Kombination eines Wilms-Tumors mit Aniridia, intersexuellem Genitale, Gonadoblastom und geistiger Retardierung wird als WAGR-Syndrom bezeichnet. Wenn Pseudohermaphroditismus und Nephropathie den Wilms-Tumor begleiten, spricht man von Drash-Syndrom. Hier werden zwei Fälle mit WAGR- und Drash- Syndromen vorgestellt und die Bedeutung der Frühdiagnose sowie die geeignete Therapie diskutiert.

Key words

Wilms' tumor - WAGR syndrome - Drash syndrome

Schlüsselwörter

Wilms-Tumor - WAGR-Syndrom - Drash-Syndrom