Volume 10,Issue 1
Fall 2025
Clinicopathological Analysis of Perivascular Epithelioid Cell Tumor in Female Genital Tract
Objective: To explore the clinicopathological features, diagnosis, and differential diagnosis of perivascular epithelioid cell tumor (PEComa) of the female genital tract. Methods: The clinical data of four patients with PEComa of the female genital tract were collected, and their histomorphological characteristics were observed. Immunohistochemical staining was performed using the EnVision two-step method, and fluorescence in situ hybridization (FISH) was used to detect TFE3 gene rearrangement. The clinicopathological features were analyzed, and relevant literature was reviewed. Results: None of the four patients were associated with tuberous sclerosis complex, and the lesions were located in the cervix, uterine body, and vaginal stump, respectively. The tumor was mainly composed of epithelioid cells arranged in solid sheets, nests, and bundles. The tumor cells had abundant cytoplasm, which was transparent or eosinophilic and granular. The nuclei showed mild or marked atypia with variable mitotic figures. Tumor necrosis, multinucleated giant cells, and rhabdoid cells were observed in some cases. Immunohistochemistry showed that HMB45, Melan A, MiTF, TFE3, desmin, SMA, and h-caldesmon were positive in three surgical specimens, while S-100 and SOX10 were negative. In one consultation case, HMB45, Melan A, desmin, and SMA were positive, while S-100 was negative. FISH detected no TFE3 gene rearrangement in three cases. Conclusion: PEComa of the female genital tract is prone to misdiagnosis. Combining typical histological, immunohistochemical, and molecular genetic features can help improve diagnostic accuracy.
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