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Perivascular epithelioid cell tumour (PEComa) of the uterus associated with malignant neoplasm of the female genital tract

case. A mass located between the upper and mid third of the posterior vaginal wall and the anterior wall of the rectum was completely excised. Total abdominal hysterectomy, bilateral salpingoophorectomy and resection of the lower part of the bowel (17 cm) with side-to-end anastomosis were performed (all removed in one block together with adjacent lymph nodes). There was no ascites, no signs of endometriosis and no hepatic, omental, ovarian or uterine pathology. The tumour was defined histologically as an adenocarcinoma, unlikely to have been of endometrial or cervical origin. Due to the uncertain origin of the tumour, an opinion was requested from three different pathology departments. All three agreed on the fact that the tumour was composed of squamous, clear cell and undifferentiated areas. The rectal mucosa, uterus, cervix, ovaries, peritoneum and lymph nodes were uninvolved. Focal adenomyosis and old fibroids were seen in the uterus. The exact origin of the tumour could not be determined, but it was suggested that it could have been endometriotic, vaginal or metastatic (from bronchus) in origin. None of these has been proven to be the origin of the tumour. The postoperative period has been uneventful, and 12 months after the operation the patient is in good health without evidence of any recurrence and without having received any further treatment.