Pediatric genitourinary tumors.

Abstract:

PURPOSE OF REVIEW:To review the 2008-2009 literature on pediatric genitourinary tumors and highlight the most significant publications. RECENT FINDINGS:New techniques such as gene expression profiling, PET, nephron-sparing surgery, and stem cell transplantation are being incorporated into contemporary treatments for pediatric patients with genitourinary tumors. The WTX gene is the most commonly mutated gene in Wilms tumor, and its product enhances Wilms tumor gene 1-mediated transcription. Germline WTX mutations cause an X-linked sclerosing bone dysplasia but do not appear to predispose to Wilms tumor formation. Protocadherin gene clusters on chromosome 5q31 may act as tumor suppressors. In rhabdomyosarcoma, ILK and platelet-derived growth factor receptor-A join the paired box gene 7 and 3-forkhead box O1 fusions as potential therapeutic targets, and muscle-specific microRNAs offer promise as adjuvant therapy. Despite the high cure rate of Wilms tumor, long-term survivors remain at risk of death from various causes. SUMMARY:In general, the prognosis for patients with pediatric genitourinary tumors is favorable. The elucidation of the molecular abnormalities in these tumors is determining risk stratification, treatment strategies, and candidates for new drug development.

journal_name

Curr Opin Oncol

authors

McLean TW,Buckley KS

doi

10.1097/CCO.0b013e32833841a1

subject

Has Abstract

pub_date

2010-05-01 00:00:00

pages

268-73

issue

3

eissn

1040-8746

issn

1531-703X

journal_volume

22

pub_type

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