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Desmoplastic small round cell tumors with EWS-WT1 fusion transcript in children and young adults

Pascale Philippe-Chomette1, Nabil Kabbara, Nicolas Andre

  • 1Department of Pediatric Surgery, Hôpital Robert Debré - Assistance Publique, Paris, France.

Pediatric Blood & Cancer
|December 14, 2011
PubMed
Abstract

Insights

Desmoplastic small round cell tumor (DSRCT) diagnosis relies on EWS-WT1 gene fusion transcript (GFT) detection. While extensive surgery showed promise, this study found no significant survival difference, emphasizing the need for complete surgical removal for long-term outcomes.

Area of Science:

  • Oncology
  • Molecular Diagnostics
  • Surgical Oncology

Background:

  • Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive cancer characterized by the EWS-WT1 gene fusion transcript (GFT).
  • Current treatment strategies for DSRCT lack standardization.
  • Accurate diagnosis is crucial for effective management.

Purpose of the Study:

  • To investigate the impact of extensive surgery on survival in young DSRCT patients.
  • To evaluate the role of EWS-WT1 GFT in DSRCT diagnosis.
  • To assess the effectiveness of different treatment modalities.

Main Methods:

  • Retrospective national multicenter analysis of patients under 30 with EWS-WT1 GFT-positive tumors.
  • Comparison of survival outcomes between limited and extensive surgical resections.
  • Analysis of initial diagnoses versus molecular testing results.

Main Results:

  • EWS-WT1 GFT was detected in 38 patients; 44.7% initially received a different diagnosis.
  • Only 24% of patients had localized disease; treatments were heterogeneous.
  • Two-year event-free survival was 14.4%, and overall survival was 50%. Four of five patients in complete remission underwent extensive, complete surgery.

Conclusions:

  • EWS-WT1 GFT detection is vital for DSRCT diagnosis.
  • No survival difference was observed based on surgical extent, but complete surgery appears beneficial for long-term survival.
  • High-dose chemotherapy and radiotherapy did not significantly improve survival in this analysis; further prospective studies are required.

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