Vulva
Malignant neoplasms
Epithelioid sarcoma

Author: Priya Nagarajan, M.D., Ph.D. (see Authors page)
Editor: Sara Peters, M.D., Ph.D.

Revised: 9 October 2017, last major update November 2014

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: "Epithelioid sarcoma" vulva

Cite this page: Nagarajan, P. Epithelioid sarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/vulvaepithelioidsarcoma.html. Accessed December 17th, 2017.
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)
Terminology
Epidemiology
  • Younger women (3rd to 5th decades)
  • Rarely reported during pregnancy
Sites
  • Vulva, including labia majora, clitoris, labia minora, mons and Bartholin glands
Clinical features
  • Usually presents as painless, rapidly enlarging tumor
Diagnosis
  • Diagnosis requires high index of suspicion and a battery of immunohistochemical stains
Radiology description
Radiology images

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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
Treatment
  • 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
Gross images

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Site unspecified

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

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Images contributed by Dr. Priya Nagarajan:
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H&E, very low power

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Low power


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Core biopsy

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Medium power



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High power

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Necrosis


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Cytokeratin

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INI1 / BAF47


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Various images, CK+, vimentin+

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Perineum

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Fig 5: various stains

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