PEComa

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PEComa
Renal angiomyolipoma (2).jpg
Histopathologic image of renal angiomyolipoma. Nephrectomy specimen. H&E stain.
MeSH D054973

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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:

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

  1. 1.0 1.1 1.2 Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F (2008). "PEComas: the past, the present and the future". Virchows Arch. 452 (2): 119–32. PMC 2234444Freely accessible. PMID 18080139. doi:10.1007/s00428-007-0509-1.  Unknown parameter |month= ignored (help)



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