PEComa

PEComa
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).

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. 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.

Diagnosis

Immunohistochemical markers

PECs typically stain for melanocytic markers (HMB-45, Melan A (Mart 1), Mitf) and myogenic markers (actin).