PURPOSE: Wilms' tumor is the most common renal tumor in children. Outcomes have improved dramatically over the past few decades, but important treatment questions remain. These include the role of molecular biologic markers in stratifying patients for therapy or targeting tumors for treatment. We present a summary of these advances and outline the current treatment of Wilm's tumor. MATERIALS AND METHODS: The medical literature and results of all cooperative group studies reporting treatment of children with Wilms' tumor were reviewed. RESULTS: Overall survival exceeds 90% for most patients with nephroblastoma. However, outcomes for patients with rhabdoid tumors and diffuse anaplasia remain poor. The role of renal sparing surgery in patients with bilateral tumors is clear, but for children with unilateral tumors it continues to be defined. CONCLUSIONS: Current protocols conducted by pediatric oncology groups are beginning to incorporate biologic features to stratify patients for therapy. Treatment strategies continue to focus on limiting late effects of treatment while maintaining an excellent survival. New therapies are needed to treat the high-risk patients who continue to have high relapse and mortality rates.
PURPOSE:Wilms' tumor is the most common renal tumor in children. Outcomes have improved dramatically over the past few decades, but important treatment questions remain. These include the role of molecular biologic markers in stratifying patients for therapy or targeting tumors for treatment. We present a summary of these advances and outline the current treatment of Wilm's tumor. MATERIALS AND METHODS: The medical literature and results of all cooperative group studies reporting treatment of children with Wilms' tumor were reviewed. RESULTS: Overall survival exceeds 90% for most patients with nephroblastoma. However, outcomes for patients with rhabdoid tumors and diffuse anaplasia remain poor. The role of renal sparing surgery in patients with bilateral tumors is clear, but for children with unilateral tumors it continues to be defined. CONCLUSIONS: Current protocols conducted by pediatric oncology groups are beginning to incorporate biologic features to stratify patients for therapy. Treatment strategies continue to focus on limiting late effects of treatment while maintaining an excellent survival. New therapies are needed to treat the high-risk patients who continue to have high relapse and mortality rates.
Authors: Linda Holmquist Mengelbier; Jenny Karlsson; David Lindgren; Ingrid Øra; Margareth Isaksson; Ildiko Frigyesi; Attila Frigyesi; Johannes Bras; Bengt Sandstedt; David Gisselsson Journal: Am J Pathol Date: 2010-09-16 Impact factor: 4.307