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Background.- Uterine sarcomas are rare tumors of uterus. They form a morphologically heterogeneous group of malignancy with aggressive nature and fatal course. Endometrial stromal sarcomas are a subgroup within uterine sarcomas with exceptional features.
Observation.- A case of endometrial stromal sarcoma is presented and the case is discussed mainly with respect to the efficiency of the preoperative diagnostic tests and the various alternatives of the therapy.