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A Rare Uterine Malignancy: A Case of High-Grade PEComa Defying Routine Immunohistochemistry
Ashish C Philip1, Garima Garima1, Swati Swati1
1Department of Pathology, University College of Medical Sciences and GTB Hospital, NEW DELHI, INDIA.
Turk Patoloji Dergisi
|December 2, 2025
Summary
Malignant uterine PEComas are rare and challenging to diagnose, especially when lacking typical markers. This case highlights TFE3-rearranged PEComas, emphasizing the need for specific markers like TFE3 for accurate diagnosis.
Area of Science:
- Oncology
- Pathology
- Gynecologic Oncology
Background:
- Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms.
- The uterus is the most common site for PEComas within the female genital tract.
- Malignant uterine PEComas are exceptionally rare and present diagnostic difficulties, particularly those lacking standard immunohistochemical (IHC) markers.
Purpose of the Study:
- To report a rare case of a high-grade, TFE3-rearranged malignant PEComa of the uterus.
- To highlight the diagnostic challenges posed by such tumors, especially when conventional markers are negative.
- To emphasize the importance of specific markers for accurate diagnosis.
Main Methods:
- Case report presentation.
- Detailed histopathological examination.
- Immunohistochemical (IHC) analysis, including TFE3 and melanocytic markers.
- Genetic analysis for TFE3 rearrangement.
Main Results:
- A high-grade, TFE3-rearranged malignant PEComa of the uterus was identified.
- The tumor lacked expression of conventional smooth muscle markers, complicating initial diagnosis.
- The characteristic nested vascular pattern was observed.
- TFE3 rearrangement was confirmed as a key diagnostic feature.
Conclusions:
- TFE3-rearranged malignant PEComas of the uterus can lack typical smooth muscle marker expression.
- These tumors can mimic other high-grade uterine malignancies.
- Recognition of the nested vascular pattern and the use of melanocytic and TFE3 markers are crucial for diagnosis.
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