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Related Experiment Videos

Pathologic features of extraosseous Ewing's sarcoma: a report from the Intergroup Rhabdomyosarcoma Study.

H Shimada1, W A Newton, E H Soule

  • 1IRS Pathology Center, Columbus Children's Hospital, OH 43205.

Human Pathology
|April 1, 1988
PubMed
Summary

Extraosseous Ewing's sarcoma (EOE) may represent a spectrum of neural tumors. Analysis of 84 cases revealed neural markers in 13 EOE tumors, suggesting a link to primitive neuroectodermal tumors.

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Area of Science:

  • Oncology
  • Pathology
  • Molecular Biology

Background:

  • Extraosseous Ewing's sarcoma (EOE) is a rare malignant bone tumor.
  • Previous studies have suggested a potential neural origin for some EOE cases.

Purpose of the Study:

  • To investigate the potential neural differentiation in extraosseous Ewing's sarcoma.
  • To classify EOE cases based on neural markers and differentiation patterns.

Main Methods:

  • Pathology review of 84 extraosseous Ewing's sarcoma cases from Intergroup Rhabdomyosarcoma Study I and II.
  • Histologic re-evaluation for rosette formation and glycogen deposition.
  • Immunohistochemical and ultrastructural studies on 14 selected tumors.

Main Results:

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  • Thirteen of 14 studied tumors showed neural markers (neuron-specific enolase, S-100 protein, neurosecretory granules).
  • EOE cases with neural markers were categorized into three subgroups based on differentiation: bidirectional neuroblastic/schwannian, monodirectional neuroblastic, and monodirectional schwannian.
  • The findings suggest EOE with neural features may be part of the peripheral primitive neuroectodermal tumor spectrum.

Conclusions:

  • Extraosseous Ewing's sarcoma exhibits neural differentiation in a subset of cases.
  • These neural EOE cases may represent a spectrum of peripheral primitive neuroectodermal tumors.
  • Further research is needed to elucidate the clinical, histopathologic, and biologic differences between neural EOE and conventional sympathetic neuroblastoma.