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Updated: May 15, 2026
07:16
Three-Dimensional Imaging of Tumor-Bearing Tissue Using the Iterative Bleaching Extends Multiplexity Approach
Published on: April 25, 2025
Perivascular Epithelioid Cell Tumors: Pathogenesis, Clinical Features, and Radiologic Challenges
Douglas Rogers1, Reham Ellessy1, Matthew Simpson1
1Department of Radiology and Imaging Sciences, University of Utah, 50 N Medical Dr, Salt Lake City, UT 84132.
Summary
Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms with diverse presentations. This review highlights their shared genetic, imaging, and histopathologic features across various locations, aiding diagnosis.
Area of Science:
- Oncology
- Pathology
- Radiology
Background:
- Perivascular epithelioid cell tumors (PEComas) are uncommon mesenchymal neoplasms with dual myomelanocytic differentiation.
- They can arise in various anatomic locations due to an unclear cell of origin.
- PEComas are often linked to mutations in TSC1, TSC2, or TFE3 genes, impacting mTOR signaling.
Purpose of the Study:
- To provide a comprehensive review of PEComas.
- To highlight shared and site-specific genetic, histopathologic, and imaging features.
- To emphasize the radiologist's role in identifying PEComas due to varied presentations and potential diagnostic overlap.
Main Methods:
- Comprehensive literature review of PEComas.
- Analysis of genetic alterations (TSC1, TSC2, TFE3 mutations).
- Correlation of histopathologic and imaging findings across different anatomic sites.
Main Results:
- PEComas exhibit shared genetic underpinnings, often involving mTOR pathway activation.
- Common imaging features include avid enhancement and fat presence, reflecting tumor angiogenesis and adiposity.
- Diverse anatomic sites show site-specific manifestations and potential imaging pitfalls.
Conclusions:
- Radiologists are crucial in recognizing PEComas due to varied presentations and overlap with common tumors.
- Understanding shared and site-specific features aids in accurate diagnosis and management.
- Targeting the mTOR pathway is critical for malignant PEComa management.
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