Difference between revisions of "PEComa"

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*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 }}
*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>
</ref>
*Clear-cell sugar tumour (CCST).
*[[clear cell sugar tumour of the lung|Clear-cell sugar tumour]] (CCST).
*Primary extrapulmonary sugar tumour.
*Primary extrapulmonary sugar tumour.


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DDx:
DDx:
*[[Clear cell sarcoma]].
*[[Clear cell sarcoma]].
*[[Melanotic Xp11 translocation renal cancer]] - in kidney.
*Other [[clear cell tumours]].
*Other [[large epithelioid tumours]].


Images:
===Images===
*[http://commons.wikimedia.org/wiki/File:Renal_angiomyolipoma_%282%29.jpg Renal AML (WC)].
<gallery>
*[http://commons.wikimedia.org/wiki/File:Lymphangioleiomyomatosis_-_very_high_mag.jpg LAM - very high mag. (WC)].
Image:Renal_angiomyolipoma_%282%29.jpg | Renal AML (WC/KGH)
*[http://commons.wikimedia.org/wiki/File:Lymphangioleiomyomatosis_-_high_mag.jpg LAM - high mag. (WC)].
Image:Lymphangioleiomyomatosis_-_very_high_mag.jpg | LAM - very high mag. (WC/Nephron)
Image:Lymphangioleiomyomatosis_-_high_mag.jpg | LAM - high mag. (WC/Nephron)
</gallery>


==IHC==
==IHC==
Line 32: Line 37:
**HMB-45.<ref name=pmid18080139/>
**HMB-45.<ref name=pmid18080139/>
**Melan A (Mart 1).
**Melan A (Mart 1).
**Mitf.  
**Mitf.<ref>{{Cite journal  | last1 = Doyle | first1 = LA. | last2 = Hornick | first2 = JL. | last3 = Fletcher | first3 = CD. | title = PEComa of the gastrointestinal tract: clinicopathologic study of 35 cases with evaluation of prognostic parameters. | journal = Am J Surg Pathol | volume = 37 | issue = 12 | pages = 1769-82 | month = Dec | year = 2013 | doi = 10.1097/PAS.0b013e31829caab3 | PMID = 24061520 }}</ref>
*Myogenic markers  
*Myogenic markers  
**Calponin.
**Calponin.
**Actin.<ref name=pmid18080139/>
**Actin.<ref name=pmid18080139/>
**Myosin.
**Myosin.
==EM==
*Premelanosomes.<ref name=pmid14629307>{{Cite journal  | last1 = Park | first1 = SH. | last2 = Ro | first2 = JY. | last3 = Kim | first3 = HS. | last4 = Lee | first4 = ES. | title = Perivascular epithelioid cell tumor of the uterus: immunohistochemical, ultrastructural and molecular study. | journal = Pathol Int | volume = 53 | issue = 11 | pages = 800-5 | month = Nov | year = 2003 | doi =  | PMID = 14629307 }}</ref>


==See also==
==See also==
*[[Tuberous sclerosis]].
*[[Tuberous sclerosis]].
*[[Melanotic Xp11 translocation renal cancer]].


==References==
==References==

Latest revision as of 15:23, 31 December 2020

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

  • Renal AML (WC/KGH)

  • LAM - very high mag. (WC/Nephron)

  • LAM - high mag. (WC/Nephron)

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. 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.
  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.
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