Endometrial Stromal Sarcoma of the Uterus with Arterial Tumor Embolus

Abstract

Endometrial stromal sarcoma (ESS) of the uterus is extremely rare. We report a case of ESS of the uterus with tumor embolus in an artery in a 36 year-old woman. The patient is a Caucasian woman who presented with vaginal bleeding. Surgical biopsy revealed low grade ESS (LGESS). Subsequently, total abdominal hysterectomy and right salpingo-oophorectomy was performed. A 3.8 cm polypoid mass was identified in the endometrial cavity, invading into the outer half of the anterior uterine wall. About 5-10% of the tumor exhibited undifferentiated sarcoma. Based on the aggressive nature of the tumor, the patient underwent left salpingo-oophorectomy and was subsequently treated with chemotherapy. She was followed up with periodic CT scans and was found to have recurrent ESS in the retroperitoneum 20 months after total hysterectomy and bilateral salpingo-oophorectomy. Rebulking surgery was not performed as the tumor showed diffuse abdominal and retroperitoneal involvement. Although LGESS may behave as an indolent tumor, it can be associated with an aggressive process and a high recurrence rate. A poor prognosis may occur when LGESS is admixed with undifferentiated components especially with evidence of arterial tumor embolus, which is herein reported.