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.[3]
  • Myogenic markers
    • Calponin.
    • Actin.[1]
    • Myosin.

EM

  • Premelanosomes.[4]

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. //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.
  3. Doyle, LA.; Hornick, JL.; Fletcher, CD. (Dec 2013). "PEComa of the gastrointestinal tract: clinicopathologic study of 35 cases with evaluation of prognostic parameters.". Am J Surg Pathol 37 (12): 1769-82. doi:10.1097/PAS.0b013e31829caab3. PMID 24061520.
  4. Park, SH.; Ro, JY.; Kim, HS.; Lee, ES. (Nov 2003). "Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study.". Pathol Int 53 (11): 800-5. PMID 14629307.