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. | ||
Revision as of 18:49, 29 January 2012
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.[1]
The PEComa family
- Angiomyolipoma.
- Lymphangioleiomyomatosis.
- Clear-cell myomelanocytic tumour of ligamentum teres/falciform ligament.
- Abdominopelvic sarcoma of perivascular epitheloid cells.[2]
- Clear-cell sugar tumour (CCST).
- Primary extrapulmonary sugar tumour.
Microscopic
Features:[1]
- Epithelioid morphology.
- Clear or granular cytoplasm.
- Central oval (or round) nucleus.
- Indistinct/small nucleolus.
DDx:
Images:
IHC
See also
References
- ↑ 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/.
- ↑ 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.