Wilms' tumor and associated malformations; report of two cases with WAGR and Drash syndrome.
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.
Ozbey
H
H
Department of Pediatric Surgery, University of Istanbul, Istanbul Faculty of Medicine, Turkey.
Boneval
C
C
Kapran
Y
Y
Aksöyek
S
S
Salman
T
T
Celik
A
A
D002675:Child, Preschool; D012734:Disorders of Sex Development / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000473:pathology; D007680:Kidney Neoplasms / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D008297:Male; D009392:Nephrectomy; D017624:WAGR Syndrome / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D009396:Wilms Tumor / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*