Perivascular Epithelioid Cell Tumors: Pathogenesis, Clinical Features, and Radiologic Challenges.

Perivascular epithelioid cell tumors (PEComas) are an uncommon group of mesenchymal neoplasms characterized by perivascular epithelioid cells that exhibit dual myomelanocytic differentiation. Due to their lack of a site-specific cell of origin, they may arise in a wide variety of anatomic locations. Although most cases are caused by sporadic mutations of TSC1, TSC2, or TFE3 genes, a subset are caused by germline mutations in patients with tuberous sclerosis complex. These genetic alterations lead to uncontrolled cell growth through overactivation of mammalian target of rapamycin, which is a critical therapeutic target for management of malignant PEComas. The authors provide a comprehensive review of PEComas, highlighting shared genetic, histopathologic, and imaging features across diverse anatomic sites, while also covering site-specific manifestations and potential imaging pitfalls. Common imaging features, such as avid enhancement and presence of fat, reflect their underlying tumor angiogenesis and adiposity within triphasic variants. Given the varied presentations and frequent imaging overlap of PEComas with more common tumors, radiologists play a crucial role in recognizing imaging features and clinical scenarios suggestive of PEComas, as management strategies often differ. ©RSNA, 2026 Supplemental material is available for this article.