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[Extraosseous Ewing's sarcoma with thoracic localization]

O Duquenne1, C H Marquette, E Cardot

  • 1Service de Pneumologie et Immunoallergologie, Hôpital A.-Calmette, CHRU, Lille.

Revue Des Maladies Respiratoires
|January 1, 1994
PubMed
Summary

Extraosseous Ewing's sarcoma can present as large thoracic tumors. Combining chemotherapy with cyclophosphamide, actinomycin D, and vincristine, along with surgical excision, shows promise for treating this rare cancer.

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Area of Science:

  • Oncology
  • Surgical Oncology
  • Pediatric Oncology

Background:

  • Extraosseous Ewing's sarcoma (EES) is a rare malignant bone tumor.
  • EES typically presents in children and young adults, often affecting the extremities, but can occur in the thoracic region.

Observation:

  • Two cases of EES presented with large thoracic tumors causing significant health decline.
  • Tumor invasion into the thoracic wall and ribs was noted in both patients.
  • Treatment strategies varied, including different chemotherapy regimens (cyclophosphamide, adriamycin, etoposide, actinomycin D, vincristine), surgical excision, and radiotherapy.

Findings:

  • One patient experienced tumor recurrence after a multi-modal treatment including cyclophosphamide and adriamycin-based chemotherapy, surgery, and radiotherapy.

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  • Another patient achieved an 80% tumor volume reduction with cyclophosphamide, actinomycin D, and vincristine chemotherapy, followed by surgery and continued chemotherapy, remaining in complete remission for 42 months.
  • The combination of cyclophosphamide, actinomycin D, and vincristine chemotherapy with surgical resection demonstrated efficacy in managing aggressive EES.

    • Implications:

    • Aggressive, multi-modal treatment is crucial for managing thoracic extraosseous Ewing's sarcoma.
    • The specific chemotherapy regimen of cyclophosphamide, actinomycin D, and vincristine, combined with surgical excision, appears to be a valuable therapeutic strategy for high-risk EES.
    • Further research is warranted to optimize treatment protocols for this rare and challenging malignancy.