PEComa: Difference between revisions
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==Overview== | ==Overview== | ||
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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> | 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> | ||
== | ==Pathophysiology== | ||
===Microscopic Pathology=== | |||
PECs consist of perivascular [[epithelioid]] cells with a clear/granular [[cytoplasm]] and central round nucleus without prominent [[nucleoli]]. | PECs consist of perivascular [[epithelioid]] cells with a clear/granular [[cytoplasm]] and central round nucleus without prominent [[nucleoli]]. | ||
== | ===Genetics=== | ||
PECs | The precursor cell of PEComas is currently unknown.<ref name=martignoni/> Genetically, PECs are linked to the [[tuberous sclerosis]] [[gene]]s [[TSC1]] and [[TSC2]]. | ||
== | ==Differentiating PEComa from other Diseases== | ||
PECs bear significant [[histologic]] and [[immunohistochemical]] similarity to: | 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.<ref name=martignoni/> | Thus, it has been advocated that the above could be classified PEComas.<ref name=martignoni/> | ||
== | ==Diagnosis== | ||
===Immunohistochemical markers=== | |||
PECs typically stain for melanocytic markers ([[HMB-45]], Melan A (Mart 1), Mitf) and myogenic markers ([[actin]]). | |||
==References== | ==References== | ||
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[[Category:Oncology]] | [[Category:Oncology]] | ||
[[Category:Oncology stub]] | [[Category:Oncology stub]] | ||
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Revision as of 14:00, 12 September 2012
PEComa | |
Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. H&E stain. | |
MeSH | D054973 |
WikiDoc Resources for PEComa |
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Evidence Based Medicine |
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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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Books |
News |
Commentary |
Definitions |
Patient Resources / Community |
Directions to Hospitals Treating PEComa Risk calculators and risk factors for PEComa
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Healthcare Provider Resources |
Continuing Medical Education (CME) |
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Business |
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]
Pathophysiology
Microscopic Pathology
PECs consist of perivascular epithelioid cells with a clear/granular cytoplasm and central round nucleus without prominent nucleoli.
Genetics
The precursor cell of PEComas is currently unknown.[1] Genetically, PECs are linked to the tuberous sclerosis genes TSC1 and TSC2.
Differentiating PEComa from other Diseases
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]
Diagnosis
Immunohistochemical markers
PECs typically stain for melanocytic markers (HMB-45, Melan A (Mart 1), Mitf) and myogenic markers (actin).
References