Uterine sarcomas in general are rare: they account for 4% of all uterine malignancies. [1][2][3][4] Endometrial sarcomas account for 10-15% of all uterine sarcomas. They make up fewer than 1% of all gynaecological malignancies. 1,2,5,6 The reported incidence is approximately one to two cases per million women in the US. 6,7 Women often present with an enlarged uterus or have complaints of heavy menstrual bleeding.Occasionally, the diagnosis is established by endometrial biopsy or diagnostic curettage. Most often, however, the diagnosis is not recognised until the time of hysterectomy. Generally, these women are pre-or peri-menopausal at the time of diagnosis. AbstractEndometrial sarcomas account for 10-15% of all uterine sarcomas. These tumours are composed of uniform cells that resemble the stromal cells of proliferative endometrium. As endometrial sarcomas are rare, accounting for less than 1% of all gynaecological malignancies, there is an absence of clinical trials and treatment guidelines do not exist. It is well-known that low-grade endometrial stromal sarcomas (LGESS) are often oestrogen-receptor-(OR) and progesterone-receptor-(PR) positive. This provides a potential target for treatment, similar to other hormonally sensitive tumours. This article reviews the literature in terms of current management strategies for LGESS, particularly the data in terms of hormonal therapy. KeywordsEndometrial stromal sarcoma, uterine sarcoma, mesenchymal tumours, hormone therapy, progestins