Vulvar "proximal-type" epithelioid sarcoma: report of a case and review of the literature

Diagn Pathol. 2013 Jul 25:8:122. doi: 10.1186/1746-1596-8-122.


Background: The "proximal-type" epithelioid sarcoma is a very rare kind of mesenchimal tumor characterized by the difficulty in histological diagnosis and the very aggressive biological behavior.

Case: We report of a case of a 63 years old woman with a vulvar "proximal-type" epithelioid sarcoma that underwent a radical surgical staging followed by an adjuvant radiotherapy. She is on follow-up care for 14 months and there is no clinical evidence of disease.

Conclusion: Even if quite rare the proximal type epithelioid sarcoma should be regarded as a separate entity of particularly aggressive biologic behaviour. Its diagnosis attracts controversies and criticism related to the surgical approach and the choice of an adjuvant therapy.

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Publication types

  • Case Reports
  • Review

MeSH terms

  • Biomarkers, Tumor / analysis
  • Biopsy
  • Female
  • Humans
  • Immunohistochemistry
  • Middle Aged
  • Neoplasm Staging
  • Radiotherapy, Adjuvant
  • Sarcoma / chemistry
  • Sarcoma / pathology*
  • Sarcoma / therapy
  • Time Factors
  • Treatment Outcome
  • Vulvar Neoplasms / chemistry
  • Vulvar Neoplasms / pathology*
  • Vulvar Neoplasms / therapy


  • Biomarkers, Tumor