Relapsed Wilms Tumor in the Context of Contemporary COG Trials
- 6 hours ago
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Wilms Tumor (WT) with favorable histology has an overall survival of >90%. However, survival drops to approximately 50% for children who experience disease relapse. There are no standardized recommendations for the timing of surgical resection at relapse, and there is currently no contemporary, multi-institutional data on the best approach for managing both distant and locoregional relapse. The ongoing Children’s Oncology Group (COG) clinical trial, AREN1921, has an important surgical aim investigating upfront vs delayed resection of relapse, but data is not expected to be published for several years. Relapsed WT can be difficult to treat for a variety of reasons. First, the primary altered molecular pathways in WT include IGF2 overexpression, wnt signaling, and microRNA processing, where targeted therapies do not exist. Patients with relapsed WT are consequently often subjected to higher intensity standard WT chemotherapeutic regimens to manage their disease. Second, local relapse can exhibit a more infiltrative and vascular encasing pattern than is typical for primary WT, which can complicate resection in a prior surgical and irradiated field. This study therefore aims to describe patterns of relapse in children with unilateral WT in the context of modern COG studies and to assess the association of relapse management strategies with survival and long-term outcomes.
Consortium PIs: Jennifer Aldrink, Richard Glick, Peter Ehrlich
Please contact Maddie Peters (Madeline.Peteres@cchmc.org) if you have any questions about how to activate the study at your PSORC site.
