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Low-grade endometrial stromal sarcoma (LG-ESS) is a rare uterine malignancy, accounting for less than 1% of all uterine malignancies. And there is no literature reporting about acute obstruction of inferior vena cava (IVC) caused by LG-ESS. In November 2021, A patient diagnosed with LG-ESS was admitted to our hospital, presenting with pitting edema of the right lower limb, menstrual disturbance, nausea, oliguria andincreased serum creatinine. Imaging suggested complete obstruction of IVC cava. This difficult case was successfully treated with staging surgery combined with endocrine therapy by a cooperative multidisciplinary team (MDT). The important role of MDT in the treatment process of such a difficult and serious case was demonstrated.
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