View source for PEComa

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

==General==
*Associated with abnormalities in TSC1 and TSC2 - the genes involved in [[tuberous sclerosis]].<ref name=pmid18080139>{{cite journal |author=Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F |title=PEComas: the past, the present and the future |journal=Virchows Arch. |volume=452 |issue=2 |pages=119–32 |year=2008 |month=February |pmid=18080139 |pmc=2234444 |doi=10.1007/s00428-007-0509-1 |url=}}</ref>

==The PEComa family==
*[[Angiomyolipoma]].
*[[Lymphangioleiomyomatosis]].
*Clear-cell myomelanocytic tumour of [[ligamentum teres]]/[[falciform ligament]].
*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 of the lung|Clear-cell sugar tumour]] (CCST).
*Primary extrapulmonary sugar tumour.

==Microscopic==
Features:<ref name=pmid18080139/>
*Epithelioid morphology.
*Clear or granular cytoplasm.
*Central oval (or round) nucleus.
**Indistinct/small nucleolus.

DDx:
*[[Clear cell sarcoma]].
*Other [[clear cell tumours]].
*Other [[large epithelioid tumours]].

===Images===
<gallery>
Image:Renal_angiomyolipoma_%282%29.jpg | Renal AML (WC/KGH)
Image:Lymphangioleiomyomatosis_-_very_high_mag.jpg | LAM - very high mag. (WC/Nephron)
Image:Lymphangioleiomyomatosis_-_high_mag.jpg | LAM - high mag. (WC/Nephron)
</gallery>

==IHC==
*Melanocytic markers 
**HMB-45.<ref name=pmid18080139/>
**Melan A (Mart 1).
**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 
**Calponin.
**Actin.<ref name=pmid18080139/>
**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==
*[[Tuberous sclerosis]].

==References==
{{Reflist}}

[[Category:Weird stuff]]