Malignant perivascular epithelioid cell neoplasm of uterus: A great mimic of fibroid

Perivascular epithelioid cell (PEC) neoplasms are an unusual group of mesenchymal tumors which arise from the perivascular epithelioid Cell that characteristically expresses melanocytic and muscle markers. These neoplasms show a wide range of morphologic spectrum and have been described in multiple anatomical locations which includes uterus. We report a 46-year-old patient diagnosed as uterine fibroid on ultrasonography in whom the hysterectomy specimen unveiled characteristic features of intramural PEComa consisting of small nests and sheets of clear epithelioid cells particularly present around the blood vessels in a background of loose oedematous stroma along with the foci of mature adipose tissue which exhibits myometrial infiltration. The correct diagnosis is crucial as there is an emerging role of mTOR inhibitors which can provide a ray of hope to the patients of PEComa.