Kevin S Buckley1. 1. Department of Pediatrics, Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157, USA. kebuckle@wfubmc.edu
Abstract
PURPOSE OF REVIEW: To review the 2009/2010 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. Biologic markers are increasingly being used to help with risk stratification of patients. WT1 mutation and 11p15 loss of heterozygosity have been associated with relapse in very low-risk Wilms tumors treated with surgery alone and may help reduce the use of chemotherapy in some children. Discussion continues on the use of fusion gene status to risk stratify alveolar rhabdomyosarcoma. Meta-analysis of the use of high-dose chemotherapy with autologous hematopoetic stem cell rescue in patients with relapsed Wilms tumor and rhabdomyosarcoma suggests that some patients may benefit more from conventional salvage chemotherapy. New agents are needed for patients with high-risk and relapsed disease to improve outcomes. 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.
PURPOSE OF REVIEW: To review the 2009/2010 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. Biologic markers are increasingly being used to help with risk stratification of patients. WT1 mutation and 11p15 loss of heterozygosity have been associated with relapse in very low-risk Wilms tumors treated with surgery alone and may help reduce the use of chemotherapy in some children. Discussion continues on the use of fusion gene status to risk stratify alveolar rhabdomyosarcoma. Meta-analysis of the use of high-dose chemotherapy with autologous hematopoetic stem cell rescue in patients with relapsed Wilms tumor and rhabdomyosarcoma suggests that some patients may benefit more from conventional salvage chemotherapy. New agents are needed for patients with high-risk and relapsed disease to improve outcomes. 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.