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Abstract
Introduction/Background Perivascular Epithelioid Cell tumors (PEComas) are classified as mesenchymal tumors. They can exhibit benign, uncertain malignant potential, or malign, and therapeutic choices vary.1 Surgery is the primary therapeutic approach.2
Methodology In this report, we present a 42-year-old pre-menopausal woman diagnosed with PEComa of uncertain malignant potential following a myomectomy.
Results Our patient was presented with abnormal uterine bleeding. Transvaginal ultrasound revealed an 85x76x70 mm type-5 leiomyoma. Cervicovaginal smear and endometrial biopsy indicated benign findings. The patient underwent a myomectomy procedure. The mass was soft and elastic unlike a leiomyoma. Pathological examination revealed the infiltration of spindle to ovoid cells with eosinophilic cytoplasm. The tumor was well demarcated and the mitotic activity was 2/50 high-power fields (HPF). Immunohistochemical analysis showed diffuse positivity for melan-A, patchy positivity for HMB-45, and positive staining for SMA, desmin, caldesmon, vimentin, and WT1 (figure 1). S100, EMA, CD1A, beta-catenin, CD10, PanCK, calretinin, inhibin, and TFE3 was negative. The Ki67 proliferation index was 2%, with no atypia, lymphovascular invasion, or necrosis observed. Based on these findings, the patient was diagnosed with PEComa: uncertain malignant potential. Following the pathology results, a thoraco-abdominopelvic CT scan was performed, revealing multiple spherical enlarged pelvic and para-aortic lymph nodes. A total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy and lymphadenectomy were performed. Pathology did not reveal any metastatic diseases.
Chemotherapy was not administered due to the uncertain malignancy potential. Pelvic radiotherapy was administered (1.8 Gy per fraction for 28 fractions, totaling 50.4 Gy) for local control. The patient was placed under three-month intervals of observation, and no signs of recurrence were detected during the first 6-month follow-up.
Conclusion In PEComa patients, preoperative findings may mimic leiomyoma or leiomyosarcoma, and definitive diagnosis can only be established through pathological examination and immunohistochemistry. Given the limited number of reported cases, there is no established standard treatment modality.
References
Folpe AL, Mentzel T, Lehr HA, et al. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol 2005;29:1558–75.
Padmehr R, Arasteh S, Aminimoghaddam S, Rahbari A, Bohloli M, Mir Eskandari SM, et al. PEComa-A rare uterine neoplasm: a case report. J Reprod Infertil. 2022;23(3):224–227. https://doi.org/10.18502/jri.v23i3.10015.
