Difference between revisions of "PEComa"

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*[[Lymphangioleiomyomatosis]].
*[[Lymphangioleiomyomatosis]].
*Clear-cell myomelanocytic tumour of [[ligamentum teres]]/[[falciform ligament]].
*Clear-cell myomelanocytic tumour of [[ligamentum teres]]/[[falciform ligament]].
*Abdominopelvic sarcoma of perivascular epitheloid cells.
*Abdominopelvic sarcoma of perivascular epitheloid cells.<ref name=pmid11406657>{{Cite journal  | last1 = Bonetti | first1 = F. | last2 = Martignoni | first2 = G. | last3 = Colato | first3 = C. | last4 = Manfrin | first4 = E. | last5 = Gambacorta | first5 = M. | last6 = Faleri | first6 = M. | last7 = Bacchi | first7 = C. | last8 = Sin | first8 = VC. | last9 = Wong | first9 = NL. | title = Abdominopelvic sarcoma of perivascular epithelioid cells. Report of four cases in young women, one with tuberous sclerosis. | journal = Mod Pathol | volume = 14 | issue = 6 | pages = 563-8 | month = Jun | year = 2001 | doi = 10.1038/modpathol.3880351 | PMID = 11406657 }}
</ref>
*Clear-cell sugar tumour (CCST).
*Clear-cell sugar tumour (CCST).
*Primary extrapulmonary sugar tumour.
*Primary extrapulmonary sugar tumour.

Revision as of 16:53, 8 December 2011

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.