Abstract
Perivascular epithelioid cell tumors, called PEComas, have a mesenchymal origin with immunoreactivity for melanocytic and smooth muscle markers. Its incidence in the form of uterine involvement is rare, between 1 and 2 cases per million inhabitants, affecting mainly women in their fifth decade of life. The present study was elaborated according to the rules of the CARE case report. The patient's medical record was analyzed, and who authorized access to it and signed the Free and Informed Consent Form, together with those involved in this work. This patient underwent treatment and medical follow-up with the supervisor of this work. Therefore, this study aimed to describe a rare clinical case report of uterine malignant perivascular epithelioid cell tumor (PEComa). Although the literature on this subject is scarce and there are no consistent criteria for diagnosis and treatment, our case in question presented aspects of unfavorable evolution (a large number of cytological atypia and high mitotic index) characterizing a PEComa with uncertain malignant potential, requiring a treatment adjuvant after surgery. The patient evolved well after the surgery and adjuvant treatment, undergoing quarterly follow-ups at the oncology and gynecology outpatient clinic, with a physical examination and nuclear magnetic resonance of the pelvis for control purposes.
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References
- Lombardi W, Lombardi LB, Ferreira MA, Silva FV, Ruas SP, Vanni FP, et al. Pecoma de colo uterino: um raro relato de caso. Relatos Casos Cir.2021;(3):e3036.
- Purwar R, Soni K, Shukla M, Verma A, Kumar T, Pandey M. TFE3-associated perivascular epithelioid cell tumor with complete response to mTOR inhibitor therapy: report of first case and literature review. World J Surg Oncol. 2022;20(1):62. Published 2022 Mar 1. doi:10.1186/s12957-021-02462-5.
- Park J, Seo AN. TFE3-expressing pri-mary perivascular epithelioid cell tumor of the Lymph node mimicking nodal relapse of rectal cancer: A case report. Int J Surg Case Rep. 2019;59:46-9. doi: 10.1016/j.ijscr.2019.05.002.
- Tobalina Bonis, Raul et al. Pecoma pélvico del ligamento ancho con metástasis ganglionares: presentación de un caso y revisión de la literatura. Arch. Esp. Urol.,2008, v. 61, n. 6, p. 744-748.
- Montalvão P, Cabrera R, Cruz J, Saraiva J, Santiago N. Pecoma cervical - a propósito de um caso clínico. Port J ORL. 2010;48(4):217-21. doi: 10.34631/sporl.215
- Theofanakis C, Thomakos N, Sotirop-oulou M, Rodolakis A. Perivascular ep- ithelioid cell tumor of the uterus: Report of two cases and mini-review of the literature. Int J Surg Case Rep. 2016;28:85-7. doi: 10.1016/j.ijscr.2016.09.017.
- Fadare O, Parkash V, Yilmaz Y, et al. Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal ‘‘PEComato-sis’’: aclinicopathological study with comparative genomic hybridization analysis. World J Surg Oncol. 2004;2:35. doi: 10.1186/1477-7819-2-35.
- Okamoto S, Komura M, Terao Y, et al. Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa). Respir Med Case Rep. 2017;22:77-82. Published 2017 Jun 27. doi:10.1016/j.rmcr.2017.06.011.
- Chan YB, Kut E, Koç A. Recurrence of retroperitoneal localized perivascular epithelioid cell tumor two years after initial diagnosis: case report. Sao Paulo Med. J. 2019;137(2):2068. doi: 10.1590/1516-3180.2017.0120050717.
- Bennett JA, Braga AC, Pinto A, et al. Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors. Am J Surg Pathol. 2018;42(10):1370-1383. doi: 10.1097/PAS.0000000000001119.