Abstract:

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.

Key words: Sarcoma, endometrial stromal, Translocation, genetic, Immunohistochemistry, Gynecologic surgical procedures, Therapy, Prognosis