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Related Experiment Video

Updated: May 10, 2026

Induction of Mesenchymal-Epithelial Transitions in Sarcoma Cells
11:42

Induction of Mesenchymal-Epithelial Transitions in Sarcoma Cells

Published on: April 7, 2017

ERG expression in epithelioid sarcoma: a diagnostic pitfall.

Markku Miettinen1, Zengfeng Wang, Maarit Sarlomo-Rikala

  • 1*Laboratory of Pathology, National Cancer Institute, Bethesda ‡Joint Pathology Center, Silver Spring, MD †HUSLab, Helsinki, Finland.

The American Journal of Surgical Pathology
|June 19, 2013
PubMed
Summary

ERG expression is common in epithelioid sarcomas, mimicking angiosarcomas. However, epithelioid sarcomas lack ERG gene rearrangement and other endothelial markers, aiding differential diagnosis. INI1/SMARCB1 loss is a key feature.

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Area of Science:

  • Oncology
  • Pathology
  • Molecular Biology

Background:

  • The ERG transcription factor is a known marker for endothelial cells, crucial for identifying angiosarcomas.
  • ERG expression also occurs in prostate carcinomas and Ewing sarcomas due to specific gene translocations.
  • Understanding ERG expression patterns is vital for accurate tumor diagnosis and classification.

Purpose of the Study:

  • To investigate the prevalence and significance of ERG expression in epithelioid sarcomas.
  • To evaluate other endothelial markers (CD31, claudin 5, Prox1, podoplanin) and INI1/SMARCB1 in epithelioid sarcomas.
  • To differentiate epithelioid sarcoma from epithelioid angiosarcoma using molecular and immunohistochemical markers.

Main Methods:

  • Immunohistochemical analysis of ERG expression in 109 epithelioid sarcoma cases.

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  • Assessment of other endothelial markers (CD31, claudin 5, Prox1, podoplanin) and INI1/SMARCB1.
  • Analysis of ERG gene rearrangement status in epithelioid sarcomas.

    • Main Results:

    • ERG was expressed in 38% of epithelioid sarcomas, showing nuclear staining similar to angiosarcomas.
    • All epithelioid sarcomas were negative for ERG gene rearrangement, distinguishing them from translocation-related ERG expression.
    • Epithelioid sarcomas generally lacked other endothelial markers (CD31, claudin 5, Prox1), but commonly expressed podoplanin and showed loss of INI1/SMARCB1.

    Conclusions:

    • ERG expression in epithelioid sarcoma can be a diagnostic pitfall, mimicking angiosarcoma.
    • The absence of ERG gene rearrangement and typical endothelial markers aids in distinguishing epithelioid sarcoma from angiosarcoma.
    • INI1/SMARCB1 loss is a characteristic feature of epithelioid sarcoma, valuable in differential diagnosis.