Parametrial angiomyolipoma with multicystic change

Angiomyolipoma is a distinctive benign neoplasm of the renal origin. Rare extrarenal instances of angiomyolipoma have been documented at various body sites. A case of extrarenal angiomyolipoma arising from the parametrial soft tissues is described in the present report.

A woman, age 40 years, presented with a large abdominopelvic mass. CT scan study revealed a solid mass with interspersed cystic areas and suggested the tumor mass to be attached in the pelvic soft tissues of the broad ligament, partly surrounding the lower uterus. These findings were corroborated by the peroperative observations. Gross examination and the microscopic evaluation of extensively sampled tumor mass and the accompanying hysterectomy specimen showed no connection of the tumor mass to the uterine wall. The tumor possessed typical histology of the angiomyolipoma and a peculiar presence of the interspersed cystic areas. Such cystic areas were epithelial lined and had morphology consistent with the paramesonephric (mullerian) cystic remnants.

Angiomyolipoma arising from the parametrial soft tissues has not been described before in the English medical literature. The paramesonephric remnants are of common occurrence in the broad ligament soft tissues. It is highly probable that such remnants are entrapped in the tumor arising from parametrial soft tissues of the broad ligament; however, probability of the tumor's origin from a paramesonephric remnant itself cannot be ruled out.