Soft tissue
Perivascular epithelioid cell
PEComa - abdominopelvic sarcoma

Topic Completed: 1 November 2012

Revised: 26 March 2019

Copyright: 2002-2018,, Inc.

PubMed Search: Abdominopelvic sarcoma

Vijay Shankar, M.D.
Page views in 2018: 410
Page views in 2019 to date: 303
Cite this page: Shankar V. PEComa - abdominopelvic sarcoma. website. Accessed October 15th, 2019.
Definition / general
  • First described in 2001 (Mod Pathol 2001;14:563)
  • Tumor masses involve serosa of ileum, uterus or pelvic cavity
  • Nodal and metastatic disease present
  • One patient had tuberous sclerosis
Case reports
Microscopic (histologic) description
  • Sheets of large polygonal cells with glycogen-rich clear or eosinophilic cytoplasm, moderately pleomorphic nuclei, delicate vasculature (resembles clear cell carcinoma)
  • Also focal coagulative necrosis and occasional mitotic figures
  • Angiolymphatic invasion present
  • May have intracytoplasmic brown pigment
  • No spindle cells, smooth muscle or fat
Positive stains
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