Thomas W McLean1, Sharon M Castellino. 1. Department of Pediatrics, Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157, USA. tmclean@wfubmc.edu
Abstract
PURPOSE OF REVIEW: We will review the recent (2006/2007) literature on pediatric genitourinary tumors. RECENT FINDINGS: The WTX gene is now the most common known gene mutation in Wilms' tumor. It acts as a tumor suppressor gene and may play a role in other malignancies. Current clinical trials have incorporated the loss of heterozygosity of 1p and 16q along with age, tumor size, stage, and response to therapy to risk stratify therapy. The PAX3-FOX01 gene fusion and the IGF-1R-signaling and mTOR-signaling pathways are implicated in rhabdomyosarcoma biology, and hence are promising therapeutic targets for this tumor. SUMMARY: Progress is being made in understanding the pathogenesis of genitourinary tumors in children, and the prognosis for these children continues to improve. Current treatment strategies are focused on improving event-free survival while decreasing late effects from therapy.
PURPOSE OF REVIEW: We will review the recent (2006/2007) literature on pediatric genitourinary tumors. RECENT FINDINGS: The WTX gene is now the most common known gene mutation in Wilms' tumor. It acts as a tumor suppressor gene and may play a role in other malignancies. Current clinical trials have incorporated the loss of heterozygosity of 1p and 16q along with age, tumor size, stage, and response to therapy to risk stratify therapy. The PAX3-FOX01 gene fusion and the IGF-1R-signaling and mTOR-signaling pathways are implicated in rhabdomyosarcoma biology, and hence are promising therapeutic targets for this tumor. SUMMARY: Progress is being made in understanding the pathogenesis of genitourinary tumors in children, and the prognosis for these children continues to improve. Current treatment strategies are focused on improving event-free survival while decreasing late effects from therapy.
Authors: Jennifer D Kubic; Kacey P Young; Rebecca S Plummer; Anton E Ludvik; Deborah Lang Journal: Pigment Cell Melanoma Res Date: 2008-12 Impact factor: 4.693