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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