Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Uterus: A Rare Type of Mesenchymal Tumors and a Management Challenge
Simple Summary
Abstract
1. Introduction
2. Materials and Methods
3. Results
3.1. Epidemiology
3.2. Clinical Presentation, Tumor Markers, and Differential Diagnosis
3.3. Imaging Features
3.4. Pathologic, Immunohistochemical, and Molecular Features
3.5. Prognosis and Malignant Potential. Risk Stratification Systems
3.6. Treatment
4. Discussion
5. Conclusions
Author Contributions
Funding
Conflicts of Interest
Abbreviations
References
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PEComa | Leiomyoma | Leiomyosarcoma | |
---|---|---|---|
US |
|
|
|
CT |
|
|
|
MRI |
|
|
|
Classification System | Benign | Uncertain Malignant Potential | Malignant |
---|---|---|---|
Folpe criteria | No worrisome features:
| One or both worrisome features:
| Two or more worrisome features:
|
Modified Folpe criteria | One or no worrisome features:
| One worrisome feature:
| Any necrosis or more than two worrisome features:
|
Schoolmeester criteria | Fewer than four worrisome features:
| Four or more worrisome features:
| |
Bennet Criteria | - | Fewer than three worrisome features:
| Three or more worrisome features:
|
WHO criteria b | - | Fewer than three worrisome features:
| Three or more worrisome features:
|
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Oliver-Perez, R.; Ortega, M.; Manzano, A.; Estrada-Lorenzo, J.M.; Martinez-Lopez, M.; Zabia, E.; Lopez-Gonzalez, G.; Madariaga, A.; Parrilla, L.; Tejerizo, A.; et al. Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Uterus: A Rare Type of Mesenchymal Tumors and a Management Challenge. Cancers 2025, 17, 2185. https://doi.org/10.3390/cancers17132185
Oliver-Perez R, Ortega M, Manzano A, Estrada-Lorenzo JM, Martinez-Lopez M, Zabia E, Lopez-Gonzalez G, Madariaga A, Parrilla L, Tejerizo A, et al. Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Uterus: A Rare Type of Mesenchymal Tumors and a Management Challenge. Cancers. 2025; 17(13):2185. https://doi.org/10.3390/cancers17132185
Chicago/Turabian StyleOliver-Perez, Reyes, Marta Ortega, Aranzazu Manzano, Jose Manuel Estrada-Lorenzo, Mario Martinez-Lopez, Elena Zabia, Gregorio Lopez-Gonzalez, Ainhoa Madariaga, Lucia Parrilla, Alvaro Tejerizo, and et al. 2025. "Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Uterus: A Rare Type of Mesenchymal Tumors and a Management Challenge" Cancers 17, no. 13: 2185. https://doi.org/10.3390/cancers17132185
APA StyleOliver-Perez, R., Ortega, M., Manzano, A., Estrada-Lorenzo, J. M., Martinez-Lopez, M., Zabia, E., Lopez-Gonzalez, G., Madariaga, A., Parrilla, L., Tejerizo, A., & Gil-Ibañez, B. (2025). Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Uterus: A Rare Type of Mesenchymal Tumors and a Management Challenge. Cancers, 17(13), 2185. https://doi.org/10.3390/cancers17132185