Abstract
Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor occurring in ~1 in 1,000,000 patients. It can arise in many parts of the body, and gynecologic PEComa accounts for a quarter of all PEComa cases. We discuss a case of a 43-year-old woman with a history of total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAHBSO) 14 years ago for uterine leiomyoblastoma, which did not require adjuvant treatment. She presented in late 2024 with vague abdominal discomfort and was subsequently found to have a left pelvic malignant PEComa.