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Introduction: Uterine mesenchymal tumors are derived from endometrial stroma, smooth muscle cells, or blood vessels. One subtype, Endometrial Stromal Sarcoma (ESS), is a rare malignant tumor that comprises approximately 1% of gynecological malignancies and less than 10% of all uterine malignancies (1). The World Health Organization classifies ESS into four subtypes: endometrial stromal nodule (ENS), low grade ESS (LG-ESS), high grade ESS (HG-ESS), and undifferentiated uterine sarcoma (UUS) due to cell morphology and mitotic counts (2). In a Surveillance, Epidemiology, and End Results (SEER) analysis of endometrial stromal sarcoma, the five-year disease-free survival was 91.4% (ENS), 95.4% (LG-ESS), and 42.1% (HG-ESS and UUS) for the respective subtypes (1). Late recurrences have been identified in LG-ESS in up to 60% of patients (3). Typical clinical presentations of ESS include the following: abnormal uterine bleeding, pelvic pressure, enlarged uterus, and abdominal distention. Diagnosis is made based upon pathology evaluation. Standard initial treatment for ESS includes a hysterectomy and bilateral salpingo-oophorectomy. There is currently no consensus on adjuvant therapy. References 1: Chan, J., Kawar, N., Shin, J. et al. Endometrial stromal sarcoma: a population-based analysis. Br J Cancer 99, 1210–1215 (2008). 2: Conklin CJ, Longacre TA. Endometrial Stromal Tumors. Advances In Anatomic Pathology. 2014; 21 (6): 383-393. 3: Tse KY, Crawford R, Ngan HY. Staging of uterine sarcomas. Best Pract Res Clin Obstet Gynaecol 2011; 25:733.