Prognostic Factors in Stage III Favorable-Histology Wilms Tumor


Key Points

  • The DD4A regimen and radiotherapy resulted in good event-free and overall survival in most patients with stage III favorable-histology Wilms tumor.
  • Positive nodes and LOH at chromosome 1p or 16q were associated with poorer event-free survival.

An analysis from the Children’s Oncology Group Study AREN0532 reported in the Journal of Clinical Oncology by Fernandez et al showed that positive lymph nodes and loss of heterozygosity (LOH) at chromosome 1p or 16q are associated with poorer event-free survival in patients with stage III favorable-histology Wilms tumor.

Study Details

The analysis included 535 patients without combined LOH at chromosome 1p and 16q who received the National Wilms Tumor Study DD4A regimen (vincristine, dactinomycin, and doxorubicin) and radiation therapy between October 2006 and August 2013 as part of the AREN0532 study.

Event-Free Survival Differences

Median follow-up was 5.2 years (range = 0.2–9.5 years). Among all patients, 4-year event-free and overall survival were 88% and 97%, respectively. Positive lymph node status (n = 151) was associated with poorer 4-year event-free survival vs negative lymph node status (n = 236; 82% vs 94%, P < .01) but not significantly poorer overall survival (96% vs 99%, P = .09). The presence of LOH at chromosome 1p (n = 56) or 16q (n = 96) vs absence of both (n = 377) was associated with poorer 4-year event-free survival (75% and 83% vs 91%; overall P < .01) but not overall survival (92% and 97% vs 97%; overall P = .21). Patients with both positive nodes and LOH at chromosome 1p or 16q had 4-year event-free survival of 73.8% (P < .001 for trend), with 4-year overall survival of 92.4% (P = .09 for trend).

The investigators concluded: “Most patients with stage III [favorable-histology Wilms tumor] had good [event-free survival]/overall survival with DD4A and radiation therapy. Combined lymph node and LOH status was highly predictive of [event-free survival] and should be considered as a potential prognostic marker for future trials.”

The study was supported by grants from the National Cancer Institute and by St Baldrick’s Foundation.

Conrad V. Fernandez, MD, of the IWK Health Centre, Dalhousie University, Halifax, is the corresponding author of the Journal of Clinical Oncology article. 

The content in this post has not been reviewed by the American Society of Clinical Oncology, Inc. (ASCO®) and does not necessarily reflect the ideas and opinions of ASCO®.




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