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Non-Rhabdomyosarcoma (Non-RMS)



Epitheliod Sarcoma (ES)



Epithelioid sarcoma (ES)

  • First described by Enzinger in 1970
  • Uncommon, accounts for 5 - 8% of malignant non-RMS in children
  • Most prevalent in young adults ages 20 – 40 years. Rarely found in children
  • Generally presents in fascial planes, aponeuroses, and tendon sheaths of the extremities, particularly in the hand (the fingers) and foot.
  • Associated with a risk of spread to regional lymph nodes



Two types - classic ES and proximal-type ES share a similar immunophenotypic profile

Classic form:

  • Distinctive nodular, granuloma-like pattern, with spindle and epithelioid cells circumscribing areas of central degeneration and necrosis.

Proximal type:

  • Rare subtype associated with more aggressive behavior and worse
  • Usually deep-seated soft tissue mass at proximal body sites
  • Sheets of large, atypical, epithelioid cells with vesicular nuclei and prominent nucleoli, with a rhabdoid phenotype.
  • Alterations of chromosome 22 with deletions of the SMARCB1/INI1 gene were recently described in this variant



  • 5-year event-free survival (EFS): just over 60%
  • 5 year overall survival (OS): just over 90%
  • OS rate dropped to 86.9% at 10 years
  • OS rate at 15 years was 72.4%

Local disease recurrence was the major cause of treatment failure.

Most significant finding influencing both EFS and OS was tumor site, with a tumor location in the extremities predicting a favorable outcome








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