Applied Immunohistochemistry in Differential Diagnosis of Female Genital System PEComas
Ema Campara1, Edina Lazovic-Salcin2, Amira Skopljak3, Merita Tiric – Campara4
1 Clinic of Oncology, Clinical Center University of Sarajevo, Sarajevo, Bosnia and Herzegovina
2 Department of Pathology, Faculty of Medicine, University of Sarajevo, Sarajevo, Bosnia and Herzegovina
3 Department of Family Medicine, Faculty of Medicine, University of Sarajevo, Sarajevo, Bosnia and Herzegovina
4 Department of Neurology, General Hospital ‘’Prim.dr. Abdulah Nakaš’’, Sarajevo, Bosnia and Herzegovina
Corresponding Author: Ema Campara, MD. Clinic for Oncology, Clinical Center University of Sarajevo, Sarajevo,
Bosnia and Herzegovina; E-mail: ema.campara@icloud.com; Phone: +38762 862-309; ORCID ID: 0009-0000-1036-7015
Cite this article: Campara E, Lazovic-Salcin E, Skopljak A, Tiric-Campara M. Applied Immunohistochemistry in
Differential Diagnosis of Female Genital System PEComas – Review Article. Sar Med J. 2025; 2(1): Online ahead
of print. 
10.70119/0025-25
Pages: -/ Published online: 01 February 2025
Original submission: 10 August 2024; Revised submission: 14 December 2024; Accepted: 28 December 2024
Abstract
PEComa (Perivascular epithelioid cell tumors) are a rare type of tumor composed of cells exhibiting characteristics of smooth muscle cells and melanocytes. They most commonly occur in the female genital system. This study is a narrative review based on the differential diagnosis of tumors in the female genital system, focusing on PEComa. The aim of the research is to analyze the immunohistochemical markers characteristic of PEComa in the female genital system and compare them with markers of tumors that may appear in the differential diagnosis. Specifically, the study examines epithelioid smooth muscle tumor (STUMP), malignant melanoma, alveolar soft part sarcoma (ASPS), poorly differentiated endometrial carcinoma (EC) and trophoblastic tumors of the placenta (PSTT). Comparison of immunohistochemical markers of PEComa with markers of other tumors revealed that: PEComas show overlap in positive staining with STUMP, but are distinguished by markers such as HMB45, PNL2, MiTF, and MelanA/MART1; PEComas share some melanocytic markers with malignant melanoma, but differ in the expression of myogenic markers and hormone receptors; compared to ASPS, PEComas share some positive staining but differ in marker expression and negative staining; they differ from EC by the expression of specific markers such as MiTF and PAX8; PSTT show specificity for markers of trophoblastic differentiation and implantation, while PEComas emphasize melanocytic and myogenic differentiation. The general conclusion is that an accurate diagnosis of PEComa in the female genital system can only be achieved through a multidisciplinary approach. Immunohistochemical evaluation serves as a helpful tool, but standard morphological staining remains the gold standard. Also, the advanced diagnostic techniques, particularly next-generation sequencing, hold promise for enhancing the understanding and management of mPEComas. By uncovering the genomic landscape and facilitating targeted therapies, these methodologies may lead to more effective treatment and improved outcomes.
.
Keywords: female genital system, epithelioid smooth muscle tumor, malignant melanoma, endometrial carcinoma, trophoblastic tumor.
.
