PEComa

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PEComa is a family of tumours derived from perivascular epithelioid cells (PECs).

General

The PEComa family

Microscopic

Features:[1]

  • Epithelioid morphology.
  • Clear or granular cytoplasm.
  • Central oval (or round) nucleus.
    • Indistinct/small nucleolus.

DDx:

Images:

IHC

  • Melanocytic markers
    • HMB-45.[1]
    • Melan A (Mart 1).
    • Mitf.
  • Myogenic markers
    • Calponin.
    • Actin.[1]
    • Myosin.

See also

References

  1. 1.0 1.1 1.2 1.3 Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F (February 2008). "PEComas: the past, the present and the future". Virchows Arch. 452 (2): 119–32. doi:10.1007/s00428-007-0509-1. PMC 2234444. PMID 18080139. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2234444/.
  2. Bonetti, F.; Martignoni, G.; Colato, C.; Manfrin, E.; Gambacorta, M.; Faleri, M.; Bacchi, C.; Sin, VC. et al. (Jun 2001). "Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis.". Mod Pathol 14 (6): 563-8. doi:10.1038/modpathol.3880351. PMID 11406657.