Perivascular epithelioid cell tumours (PEComas)

A.Prof Frank Gaillard et al.

Perivascular epithelioid cells tumours (PEComas) are a group of related mesenchymal tumours and tumour-like conditions found in many locations. This group includes:

Epidemiology

PEComas are far more common in females 4

Associations

Pathology

Histology

All PEComas share similar and distinctive cellular morphology – peripheral epitheloid cells – which have no counterpart in normal tissue 4. These tumours appear as sheets and nests of epithelioid or spindle-shaped cells associated with blood vessel walls. They have granular eosinophilic cytoplasms. 

Immunohistochemistry

PEComas are characterised by immunoreactivity to smooth muscle and melanocytic markers 4.

  • smooth muscle: actin, desmin
  • melanocytic: HMB-45, melan-A
Treatment and prognosis

PEComas usually behave in a benign fashion although local recurrence and even malignant behaviour are encountered, mimicking malignant sarcomas. 

Surgical excision is usually curative. 

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Article information

rID: 9985
Tags: cases, refs
Synonyms or Alternate Spellings:
  • Perivascular epithelioid cell tumours (PEComa)
  • PEComa
  • PEComas
  • Perivascular epithelioid cell tumours (PEComas)
  • Perivascular epithelioid cell tumors (PEComa)
  • Perivascular epithelioid cell tumor (PEComa)
  • Peri-vascular epithelioid cell tumor (PEComa)

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