Article type :

Case Report

Author :

Raghav Kapoor*, Anila Sharma, Sunil Pasricha, Meenakshi Kamboj, Anurag Mehta

Volume :

11

Issue :

1

Abstract :

The term “Perivascular epithelioid cells” was described as early as 1992. WHO has defined PEComas as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. These tumors have a varied morphology including sheets and nested pattern comprising of epithelioid to spindle tumor cells with increased vasculature often in a sinusoidal pattern. PEComas show immunoreactivity for melanocytic and smooth muscle markers, however small number cases show lack of muscle marker expression along with strong TFE3 nuclear positivity. Here we describe a PEComa of soft tissue of leg, TFE3 rearranged.