Abstract
A perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm with distinctive perivascular epithelioid cells that usually demonstrates myomelanocytic differentiation. PEComas can arise in various organs and generally are benign. Uncommonly PEComas have been documented to be malignant with metastasis most frequently to the lung, liver, lymph nodes, and bone. Here, we present the case of a 59-year-old male with a malignant retroperitoneal PEComa with confirmed metastasis to the femur and suspected metastasis to the liver and lung. The purpose of this case study is to present the progression and findings of a metastatic malignant PEComa.