INTRODUCTION: Perivascular epithelioid cell tumors (PEComa), derived from perivascular epithelioid cells (PEC), are a group of mesenchymal tumors, as defined by the World Health Organization. PEComas of the lung are also known as Clear Cell Sugar tumor (CCTL). They’re typically reactive to smooth muscle and melanocytic markers. The literature shows that while PEComas may not have a clear sexual predominance, they are likely more common in females. They are also mainly found in middle aged adults however they can be prevalent in patients from 8 to 73 years of age (Bleeker et al., 2012) (Yan et al., 2024)PEComas are generally considered benign (Yan et al., 2024), however there is a potential subset with malignant behavior. In this article, we report a potential case of malignant PEComa of the lung in a female patient and we discuss the molecular profile of this tumor.
