PEComa: Difference between revisions
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==Overview== | ==Overview== | ||
In [[oncology]], '''PEComa''', also '''PEC tumour''' and '''perivascular epithelioid cell tumour''', is a type of | In [[oncology]], '''PEComa''', also '''PEC tumour''' and '''perivascular epithelioid cell tumour''', is a type of mesenchymal tumour consisting of perivascular epithelioid [[cell (biology)|cell]]s (PECs).<ref name=martignoni>{{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> | ||
==Histologic appearance== | ==Histologic appearance== |
Revision as of 17:47, 7 March 2009
PEComa | |
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Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. H&E stain. | |
eMedicine | orthoped/377 |
MeSH | D054973 |
WikiDoc Resources for PEComa |
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Ongoing Trials on PEComa at Clinical Trials.gov Clinical Trials on PEComa at Google
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US National Guidelines Clearinghouse on PEComa
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Definitions |
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Directions to Hospitals Treating PEComa Risk calculators and risk factors for PEComa
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Experimental / Informatics |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
In oncology, PEComa, also PEC tumour and perivascular epithelioid cell tumour, is a type of mesenchymal tumour consisting of perivascular epithelioid cells (PECs).[1]
Histologic appearance
PECs consist of perivascular epithelioid cells with a clear/granular cytoplasm and central round nucleus without prominent nucleoli.
Immunohistochemical markers
PECs typically stain for melanocytic markers (HMB-45, Melan A (Mart 1), Mitf) and myogenic markers (actin).
PECs and other conditions
PECs bear significant histologic and immunohistochemical similarity to:
- angiomyolipoma,
- clear-cell sugar tumour (CCST),
- lymphangioleiomyomatosis, and,
- clear-cell myomelanocytic tumour of ligamentum teres/falciform ligament.
Thus, it has been advocated that the above could be classified PEComas.[1]
Etiology
The precursor cell of PEComas is currently unknown.[1] Genetically, PECs are linked to the tuberous sclerosis genes TSC1 and TSC2.
References