Soft tissue
Perivascular epithelioid cell
Sclerosing PEComa

Author: Vijay Shankar, M.D.

Revised: 30 January 2018, last major update November 2012

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Sclerosing PEComa

See also: PEComa - general

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Cite this page: Shankar, V. Sclerosing PEComa. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuepecgeneralsclerosing.html. Accessed April 21st, 2019.
Definition / general
  • Variant with extensive stromal hyalinization first described in 2008 (Am J Surg Pathol 2008;32:493)
  • Women, mean age 49 years (range 34 - 73 years)
  • 77% in retroperitoneum, usually pararenal
Case reports
Treatment
  • Excision; may recur / metastasize if high grade morphology
Gross description
  • Median 10 cm, well circumscribed
Gross images

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Well-circumscribed,
encapsulated tumor

Microscopic (histologic) description
  • Cords and trabeculae of bland, uniform, epithelioid cells with pale eosinophilic, granular to clear cytoplasm and round nuclei with small nucleoli
  • Tumor cells are arranged at least focally around blood vessels
  • Abundant densely sclerotic stroma
  • Often has spindle cell component
  • No / rare mitotic figures; rarely necrosis
  • Rarely non necrotizing granulomas, dystrophic calcifications
Microscopic (histologic) images

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Nests of clear cells in sclerotic stroma

Various stains

Positive stains
Negative stains
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