A BSTRACT Uterine sarcomas account for approximately 1% of all female genital tract malignancies and 3%–7% of all uterine cancers. Endometrial stromal sarcoma (ESS) is a rare malignant tumor of the endometrium, mostly occurring in premenopausal women. Most of these tumors are diagnosed retrospectively. In this case report, we describe a case of a 40-year-old premenopausal woman who presented with complaints of heavy menstrual bleeding and generalized weakness for the past 8 months. Clinical examination and investigations showed a uniformly enlarged uterus, and ultrasonography showed a large uterine fibroid. Patient underwent a total abdominal hysterectomy with bilateral salpingectomy. Histopathological examination revealed a high-grade ESS (HG-ESS). The patient was evaluated further with IHC studies, which also revealed it as HG-ESS. The patient was given radiotherapy postoperatively and is on regular follow-up.
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