Abstract: Endometrial stromal sarcoma is origin from the mesenchymal tissue of uterine sarcoma. The cells of it resemble normal endometrial stromal cells in proliferative stage with strong invasiveness. It can be divided into three kinds including of low grade malignant endometrial stromal sarcoma，high grade malignant endometrial stromal sarcoma and extrauterine endometrial stromal sarcoma. Therefore，early diagnosis and treatment is very important. However， endometrial stromal sarcoma cells with multiple differentiation potential， endoscopic manifestations of complex and diverse， often lack the specificity of clinical symptoms. The rates of misdiagnosis and missed diagnosis are very high. It is necessary to understand the endometrial stromal sarcoma comprehensively.

Key words: Sarcoma, endometrial stromal, Endometrial stromal tumors, Diagnostic errors, Receptors, estrogen, progesterone