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Abstract：
Endometrial stromal sarcoma (ESS) occurs rarely and accounts for only 0.2% of all uterine malignancies. ESS usually expresses estrogen and progesterone receptors, and is regarded as hormone-sensitive. Due to the rarity of these tumors, there are only few case series on the use of aromatase inhibitors in the treatment of low-grade ESS. The present study reports the cases of two patients with residual or recurrent low-grade ESS who experienced long-term disease-free survival following treatment with letrozole. The study also reviews the literature with regard to the data on aromatase inhibitors used in patients with low-grade ESS. In total, 30 patients with recurrent or residual low-grade ESS who were treated with aromatase inhibitors were identified, including the present cases. Among the 30 patients, the overall response rate of advanced low-grade ESS to aromatase inhibitors was 77.4% (complete response, 25.8%; partial response, 51.6%) and the disease control rate was 90.3%. The response rate of first-line treatment was similar to that of second-line therapy or higher (84.6 vs. 72.2%; P=0.453). Duration of aromatase inhibitor treatment ranged from 1.5 to 168 months (median, 26.5 months). The aromatase inhibitors showed minimal adverse effects. In conclusion, aromatase inhibitors, particularly third-generation drugs, are a well-tolerated class of medications that are effective in the treatment of advanced low-grade ESS, with a favorable toxicity profile.
摘要：
子宫内膜间质肉瘤（ESS）很少发生，仅占所有子宫恶性肿瘤的0.2％。ESS通常表达雌激素和孕激素受体，并被认为是激素敏感的。由于这些肿瘤的稀有性,只有少数病例系列使用芳香化酶抑制剂治疗低级别ESS。本研究报告了两名患有残留或复发性低度ESS的患者在使用来曲唑治疗后经历了长期无病生存的情况。该研究还回顾了有关低级别ESS患者使用的芳香化酶抑制剂数据的文献。总的来说,确定了30例复发或残留的低度ESS患者接受芳香化酶抑制剂治疗，包括目前的情况。在30名患者中，高级低级别ESS对芳香化酶抑制剂的总体反应率为77.4%(完全反应，25.8%；部分反应，51.6%),疾病控制率为90.3%。一线治疗的反应率与二线治疗相似或更高（84.6vs.72.2%；P=0.453）。芳香化酶抑制剂治疗的持续时间为1.5至168个月(中位数，26.5个月）。芳香酶抑制剂显示最小的副作用。总之,芳香化酶抑制剂，特别是第三代药物，是一类耐受性良好的药物，可有效治疗晚期低度ESS，具有良好的毒性。
