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Surgical Treatment of an Endolymphatic Sac Tumor
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Bilateral Wilms Tumor: A Surgical Perspective.

Andrew J Murphy1,2, Andrew M Davidoff3,4

  • 1Department of Surgery, St. Jude Children's Research Hospital, Memphis, TN 38105, USA. andrew.murphy@stjude.org.

Children (Basel, Switzerland)
|September 26, 2018
PubMed
Summary

Standardized neoadjuvant chemotherapy and nephron-sparing surgery improve outcomes for bilateral Wilms tumor (BWT). However, optimizing surgical timing and coordination is crucial for minimizing long-term toxicities in BWT management.

Keywords:
bilateral Wilms tumornephron-sparing surgery

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Area of Science:

  • Pediatric Oncology
  • Surgical Oncology
  • Nephrology

Background:

  • Bilateral Wilms tumor (BWT) historically had non-standardized management, leading to poor renal and oncologic outcomes.
  • Risk of end-stage renal disease necessitates balancing oncologic control with renal preservation.
  • Neoadjuvant chemotherapy and nephron-sparing surgery are now guiding principles for BWT management.

Purpose of the Study:

  • To review the surgical management strategy for bilateral Wilms tumor.
  • To emphasize the impact of surgical decision-making timing on disease outcomes and long-term toxicities.
  • To highlight the need for collaboration between oncologists and surgeons in BWT treatment.

Main Methods:

  • Review of current management principles for bilateral Wilms tumor.
  • Discussion of disease biology and its influence on surgical strategy.
  • Analysis of treatment timepoints impacting surgical decision-making.

Main Results:

  • A Children's Oncology Group study (AREN0534) confirmed benefits of standardized neoadjuvant chemotherapy and nephron-sparing surgery.
  • Less than 50% of BWT patients underwent bilateral nephron-sparing surgery in the AREN0534 study.
  • Coordination of chemotherapy and surgical timing is key but challenging.

Conclusions:

  • Standardized neoadjuvant chemotherapy and nephron-sparing surgery improve outcomes in BWT.
  • Optimizing the timing and implementation of bilateral nephron-sparing surgery is critical.
  • Collaborative decision-making between oncologists and surgeons is essential for minimizing long-term toxicities in BWT patients.