Malignant uterine/broad-ligament perivascular epithelioid cell tumor (PEComa) coexisting with leiomyoma in a postmenopausal woman: A case report

Perivascular epithelioid cell tumor of the uterus is a rare mesenchymal neoplasm with variable malignant potential and nonspecific clinical and radiologic features, often mistaken preoperatively for leiomyoma. This report describes the case of a postmenopausal woman with seven months of persistent postmenopausal bleeding, pelvic pain, and symptomatic anemia. Pelvic ultrasound and magnetic resonance imaging demonstrated a markedly enlarged, irregular uterus with multiple masses interpreted as fibroids. The patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy for refractory bleeding and concern for occult malignancy. Histopathological examination revealed coexisting benign leiomyomas and a malignant perivascular epithelioid cell tumor involving the uterine wall with extension into the broad ligament. The tumor showed epithelioid morphology with brisk mitotic activity, necrosis, and dual myomelanocytic differentiation on immunohistochemistry (HMB-45 and desmin positive). Whole-body positron emission tomography demonstrated no metastatic disease. Complete resection followed by adjuvant radiotherapy was associated with resolution of bleeding and pain. This case highlights that malignant uterine perivascular epithelioid cell tumor can mimic benign leiomyoma, may coexist with a true leiomyoma, and should be considered in women with persistent postmenopausal bleeding and an enlarged fibroid-appearing uterus.