Soft tissue
Perivascular epithelioid cell
PEComa - abdominopelvic sarcoma

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 22 September 2016, last major update November 2012

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PubMed Search: Abdominopelvic sarcoma

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
Micro 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
Micro Images

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Large neoplastic cells

Abundant cytoplasm

Nodal metastasis

Virtual Slides

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Large renal tumor

Positive Stains