Format

Send to

Choose Destination
Urologe A. 2007 Jun;46(6):671-4.

[Multicystic renal tumor in a patient with WAGR syndrome].

[Article in German]

Author information

1
Urologische Klinik , Lehrkrankenhaus der Universitätsklinik Charité zu Berlin, Carl-Thiem-Klinikum Cottbus GmbH, Thiemstrasse 111, 03048 Cottbus, Germany.

Abstract

The WAGR syndrome is a combination of Wilms' tumor, aniridia, genitourinary anomalies, and mental retardation. We report on a 2-year-old boy, who had a deletion of the aniridia gene PAX6 and the Wilms' tumor gene 1 (WT1 gene). At the age of 23 months, a 1.7 x 1.9 cm-sized intrarenal tumor was detected by ultrasound examination. According to the protocol of the SIOP study, a cycle of neoadjuvant chemotherapy was prescribed followed by a left-sided nephrectomy. However, postsurgical histomorphology failed to confirm the suspected diagnosis of Wilms' tumor and instead revealed dysgenetic cysts of the kidney. Based on the image morphology in connection with the deletion of the WT1 gene, the tentative diagnosis of a nephroblastoma had to be made. The study protocol of the SIOP does not permit another therapy algorithm.

PMID:
17295033
DOI:
10.1007/s00120-007-1303-z
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Springer
Loading ...
Support Center