Extraskeletal Ewing's sarcoma: a report of 18 cases and literature review

Chin J Cancer. 2010 Apr;29(4):420-4. doi: 10.5732/cjc.009.10402.

Abstract

Background and objective: Extraskeletal Ewing's sarcoma (EES) is a rare, rapidly growing, round-cell, malignant tumor that can develop in the soft tissues at any location. This study was to analyze the clinical features, diagnosis and treatment of EES.

Methods: Clinical data of 18 patients with EES, treated at between Cancer Center of Sun Yat-sen University between 1995 and 2007, were analyzed.

Results: Of the 18 patients, 13 were male and 8 were female, aged from 8 months to 60 years. Twelve (66.7%) patients were between 5-25 years of age. Eight (44.4%) patients had tumors originated from low extremities.Sixteen patients had masses at their first visit. Sixteen patients were treated by the combined modality therapy, and 2 patients were treated by the single modality therapy. The 1-, 3- and 5- year actuarial survival rates were 82.4%, 64.2% and 32.1%, respectively. The presence of metastatic disease at the time of diagnosis and the mode of treatment were prognostic factors.

Conclusions: EES is common in adolescent. It often manifests as a localized mass. The combined modality therapy is recommended for this disease. The presence of metastatic disease at the time of diagnosis and the mode of treatment are prognostic factors.

Publication types

  • Review

MeSH terms

  • 12E7 Antigen
  • Adolescent
  • Adult
  • Antigens, CD / metabolism
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Bone Neoplasms / secondary
  • Cell Adhesion Molecules / metabolism
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Female
  • Humans
  • Infant
  • Lower Extremity
  • Lung Neoplasms / secondary
  • Male
  • Middle Aged
  • Neoplasm Recurrence, Local
  • Neoplasm, Residual
  • Radiotherapy, High-Energy
  • Sarcoma, Ewing / diagnosis
  • Sarcoma, Ewing / metabolism
  • Sarcoma, Ewing / pathology
  • Sarcoma, Ewing / surgery
  • Sarcoma, Ewing / therapy*
  • Soft Tissue Neoplasms / diagnosis
  • Soft Tissue Neoplasms / metabolism
  • Soft Tissue Neoplasms / pathology
  • Soft Tissue Neoplasms / surgery
  • Soft Tissue Neoplasms / therapy*
  • Survival Rate
  • Vimentin / metabolism
  • Young Adult

Substances

  • 12E7 Antigen
  • Antigens, CD
  • CD99 protein, human
  • Cell Adhesion Molecules
  • Vimentin