Normally, no perivascular epitheloid cells exist; the name refers to the characteristics of the tumour when examined under the microscope.

The genes responsible for this multi-system genetic disease have also been implicated in other PEComas.

[2] Many PEComa types shows a female predominance in the sex ratio.

[4] PECs consist of perivascular epithelioid cells with a clear/granular cytoplasm and central round nucleus without prominent nucleoli.

PECs bear significant histologic and immunohistochemical similarity to: Thus, it has been advocated that the above could be classified PEComas.