Endometrial stromal sarcoma (ESS) represents a very rare pathological entity occurring as a malignant disease in women genital sphere. Our clinical report is based on a group of four women aged 37, 48, 50 and 70-year-old, that have been histologically diagnosed with endometrial stromal sarcoma. The most common symptom sending the patient to the physician has been the vaginal bleeding, occurring in all patients. Other associated symptoms were the abdominal enlargement and the presence of the pelviabdominal mass generated by the tumor, low to medium abdominal pain or polakiuria. Two patients were diagnosed with ESS after accomplishing a biopsic curettage of the uterus. Total abdominal hysterectomy and salpingo-oophorectomy have been successfully performed for all of the patients. Adjuvant therapy-radiotherapy has been administered to three patients. At this time, none of the patients died of the disease. Our paper also includes a concise review of the literature in order to have an up-to-date conception regarding diagnosis, therapy and outcome for ESS.

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