Abstract
The tumors known as "PEComas" or perivascular epithelioid cell tumors are a group of lesions composed exclusively or predominantly of epithelioid cells. These cells have abundant clear to eosinophilic granular cytoplasm and are HMB-45 positive. Other members of this family are some of the forms of angiomyolipoma and lymphangioleiomyomatosis, as well as clear-cell "sugar" tumor. We describe a case of these features localized in the uterus of a 34- year-old female. She was operated on with the suspicion of uterine myoma; the histological examination revealed perivascular epithelioid cell tumor (PEComa). A total hysterectomy and bilateral salpingo-oophorectomy was performed. Because of the extremely small number of cases, PEComas are considered of uncertain malignant potential. We also discuss the possibility of considering PEComa as an independent entity or whether it has to be classified as an unusual variety of epithelioid smooth-muscle neoplasm. (J GYNECOL SURG 21:161)
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