Perivascular Epithelioid Cell Neoplasm of the Uterus: A Case Report

ABSTRACT Perivascular epithelioid cell neoplasms (PEComas) represent a rare category of mesenchymal tumors, with the gastrointestinal and gynecological systems being the most frequently affected sites. A 46‐year‐old patient was referred to our clinic due to prolonged menstrual bleeding, without additional symptoms. Histopathological analysis of the specimens collected through exploratory curettage demonstrated the presence of uterine PEComa, characterized by positive immunohistochemical markers including HMB‐45, TFE3, Cathepsin K, and ER. The patient underwent laparoscopic hysterectomy with bilateral adnexectomy. No recurrence of the disease was observed during the subsequent examinations. This case underscores a common diagnostic pitfall: the absence of a distinct mass on imaging. It highlights that a PEComa can present with only non‐specific endometrial thickening, making preoperative diagnosis exceptionally challenging and reliant on histopathology. Laparoscopic surgery in our case provided the patient with quick recovery and a less invasive approach with satisfying results and the absence of recurrent disease during a one‐year follow‐up period. Future multicenter studies should facilitate a deeper comprehension and undoubtedly lead to a more effective management of a rare and enigmatic condition that is uterine PEComa.