低级别子宫内膜间质肉瘤

Low-Grade Endometrial Stromal Sarcoma

低级别子宫内膜间质肉瘤(low-grade endometrial stromal sarcoma,LG-ESS)是一类罕见的间叶源性恶性肿瘤,也是子宫内膜间质肉瘤最常见的亚型。LG-ESS作为遗传异质性疾病具有许多特征性的染色体易位,融合基因表达的异常融合蛋白是LG-ESS发展的重要驱动因素,同时也为其提供了潜在的治疗靶点。LG-ESS为低度恶性肿瘤,以生长缓慢、晚期复发为特征,骨盆辐射和长期使用他莫昔芬或雌激素为其重要危险因素。由于临床表现的不典型性,目前尚缺乏有效的术前诊断,形态学及联合应用免疫组织化学抗体有助于提高检出率,但价值有限。因此其诊治对于临床医师而言,仍是一个巨大挑战。综述LG-ESS的分子机制、临床表现、病理诊断、治疗及预后的研究进展。

Low-grade endometrial stromal sarcoma(LG-ESS)is a rare type of malignant tumor of mesenchymal origin,which is also known as the most common subtype of endometrial stromal sarcoma.As a genetically heterogeneous disease,LG-ESS has many characteristic chromosomal translocations.Abnormal fusion proteins expressed by fusion genes are an important driving factor for the development of LG-ESS and also provide potential therapeutic targets for LG-ESS.LG-ESS is a low-grade malignant tumor characterized by slow growth and late recurrence,with pelvic radiation and long-term use of tamoxifen or estrogen as important risk factors.Due to the atypial of clinical manifestations,there is still a lack of effective preoperative diagnosis.Morphology and combined application of immunohistochemical antibodies can improve the detection rate,but their value is limited.Therefore,its diagnosis and treatment remains a great challenge for clinicians.This article reviews the molecular mechanisms,clinical manifestations,pathological diagnosis,treatment and prognosis of LG-ESS.