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Pediatric chest wall Ewing's sarcoma.

N C Saenz1, D J Hass, P Meyers

  • 1Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY, USA.

Journal of Pediatric Surgery
|April 19, 2000
PubMed
Summary
This summary is machine-generated.

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Primitive neuroectodermal tumors (PNET) of the chest wall, also known as Ewing's sarcoma (ES), can be treated with surgery and chemotherapy. Long-term survival is possible, even without radiation therapy for these rare chest wall tumors.

Area of Science:

  • Pediatric Oncology
  • Thoracic Surgery
  • Sarcoma Research

Background:

  • Primitive neuroectodermal tumors (PNET) of the chest wall, including Ewing's sarcoma (ES), are rare malignancies with historically poor prognoses.
  • Multimodality treatment approaches have improved survival rates, but the role of adjuvant radiation therapy remains uncertain.

Purpose of the Study:

  • To evaluate the outcomes of patients with primitive neuroectodermal tumors (PNET) of the chest wall treated with multimodality therapy.
  • To determine the impact of neoadjuvant chemotherapy and adjuvant radiation therapy on survival in patients with chest wall PNET-ES.

Main Methods:

  • Retrospective analysis of 20 consecutive patients with PNET-ES of the chest wall treated between 1979 and 1998.
  • Patients received varying treatment regimens including neoadjuvant chemotherapy, chest wall resection, and adjuvant chemotherapy/radiation therapy.

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Main Results:

  • Eleven of 20 patients (55%) achieved long-term disease-free survival (median 7.5 years).
  • Neoadjuvant chemotherapy followed by resection was associated with better survival (7 of 11 survivors).
  • Long-term survival was achieved in patients both with and without radiation therapy, suggesting its role may be selective.

Conclusions:

  • Complete chest wall resection, potentially facilitated by neoadjuvant chemotherapy, offers a possibility for long-term survival in ES-PNET.
  • Adjuvant radiation therapy is not uniformly necessary, and its use should be individualized based on patient-specific factors.
  • Further research is needed to refine treatment strategies for these rare pediatric tumors.