Vulva, vagina & female urethra
Other tumors
Epithelioid sarcoma

Topic Completed: 1 November 2014

Minor changes: 31 May 2020

Copyright: 2002-2019,, Inc.

PubMed Search: "Epithelioid sarcoma" vulva

Priya Nagarajan, M.D., Ph.D.
Sara B. Peters, M.D., Ph.D.
Page views in 2019: 1,524
Page views in 2020 to date: 1,186
Cite this page: Nagarajan P, Peters SB. Epithelioid sarcoma. website. Accessed August 6th, 2020.
Definition / general
  • Epithelioid sarcomas (ES) are rare, slow growing sarcomas with a protracted clinical course that typically occur in distal extremities of young adults, see Soft tissue chapter
    • First described in 1970 by Enzinger as epithelioid or spindled cells in a multilobular granuloma-like pattern with central hyalinization or necrobiosis (Cancer 1970;26:1029)
  • Proximal type epithelioid sarcomas (PES), which occur in vulva, were later described as aggressive tumors occurring in proximal body sites, which tend to be composed exclusively of epithelioid cells, often with rhabdoid morphology (Am J Surg Pathol 1997;21:130)
  • Younger women (3rd to 5th decades)
  • Rarely reported during pregnancy
  • Vulva, including labia majora, clitoris, labia minora, mons and Bartholin glands
Clinical features
  • Usually presents as painless, rapidly enlarging tumor
  • Diagnosis requires high index of suspicion and a battery of immunohistochemical stains
Radiology description
Radiology images

Images hosted on other servers:
Missing Image

Vulvar mass

Prognostic factors
  • Tumor associated mortality of 75 - 100% but early surgical resection with adequate margins may lead to a better outcome
  • No definite prognostic indicators identified to date
  • Local recurrence is common despite negative surgical margins
  • 60 - 75% develop distant metastasis
Case reports
  • Surgical resection with adequate margins and regional (inguinal and pelvic) lymph node biopsy or dissection
  • Chemotherapy and radiotherapy
Gross description
  • Single lesion or multiple nodules, with variable satellite-like extensions
  • Typically in deep dermis and subcutaneous tissues
  • Soft and fleshy to firm, with focal hemorrhage and necrosis
Microscopic (histologic) description
  • Classic features of epithelioid sarcoma (epithelioid and spindled cells in a granulomatous pattern with central hyalinization, necrobiosis or necrosis), are not commonly seen in PES / MRT
  • Cells are usually polyhedral and epithelioid with abundant eosinophilic cytoplasm, sometimes with dense accentuation resulting in rhabdoid morphology
  • Focal aggregates of plump spindled cells may also be seen
  • Nuclei are large, circular or ovoid, with vesicular chromatin and prominent nucleoli
  • Pleomorphic cells with multiple nuclei may also be present
  • Mitoses are frequent and atypical mitotic figures may be identified
  • Individual cell necrosis and geographic necrosis, away from blood vessels, are common features
Microscopic (histologic) images

Images hosted on other servers:

Images contributed by Dr. Priya Nagarajan:
Missing Image

H&E, very low power

Missing Image Missing Image Missing Image Missing Image Missing Image

Low power

Missing Image

Core biopsy

Missing Image Missing Image Missing Image Missing Image

Medium power

Missing Image Missing Image Missing Image

High power

Missing Image Missing Image


Missing Image Missing Image


Missing Image

INI1 / BAF47

Missing Image Missing Image

Various images, CK+, vimentin+

Missing Image


Cytology description
  • Fine needle aspiration often yields clusters of cells with similar morphology
Positive stains
Focal positivity
Negative stains
Electron microscopy description
  • EM findings are nonspecific and include abundant cytoplasm with well developed rough endoplasmic reticulum, increase in free ribosomes, mitochondria, glycogen granules, lysosomes and fat droplets
  • Aggregates of intermediate filaments are also seen, corresponding to rhabdoid inclusions
  • In other cells, short bundles of intermediate filaments are scattered beneath the plasma membrane or around the nuclei (Pathol Int 2004;54:616, Am J Surg Pathol 1997;21:130)
Molecular / cytogenetics description
Back to top