Systemic therapy for endometrial stromal sarcomas: current treatment options

Abstract

Uterine endometrial stromal sarcomas including true low-grade endometrial stromal sarcoma (LG-ESS) and high-grade (HG-ESS) or undifferentiated endometrial sarcoma (UES) constitute a group of rare, aggressive malignancies. Most LG-ESSs express steroid receptors. Surgery is the principal primary therapy for endometrial stromal sarcomas and should be considered in all cases. These malignancies are relatively radio-and chemoresistant. Chemotherapy is used in recurrent and advanced HG-ESS and UES. Currently, the combination of gemcitabine and docetaxel is considered the most effective regimen, but at the expense of substantial toxicity. In steroid receptor positive advanced LG-ESS hormonal therapy, mainly with progestins, allows in some patients for a long-term survival. Aromatase inhibitors seem to be equally effective as first-and subsequent-line of treatment, and are well tolerated. The role of molecular-targeted therapies in endometrial stromal sarcomas remains to be established.

Endometrial Stromal Sarcoma Arising from Endometriosis

“…Surgery consisting of total hysterectomy and bilateral salpingo-oophorectomy is the principal therapy of early ESSs; whereas, the role of elective lymphadenectomy is not well established. Ovary-sparing surgery in young women with early ESS is a matter of debate, as this procedure is associated with relatively high risk of local recurrence (24).…”

“…Progestins are used as the first-line treatment, after prior chemotherapy and as an adjunct to complete resection of metastatic lesions (24).…”

“…The effect of tamoxifen on ESS has also not been well studied, but case reports have suggested that tamoxifen promotes progression of ESS acting as a weak estrogen in the gynecologic tract (20–39).…”

“…Currently, the third-generation aromatase inhibitors (AIs) and GnRH analogues have become alternative endocrine treatments for ESS, but data on the efficacy of these drugs for ESS are scarce, and most published studies are only case reports. Responses reported are usually partial even if complete responses are also reported (24).…”

Aromatase inhibitor therapy for endometrial stromal sarcoma — two-centre experience

“…Endometrial stromal tumors are rare entities, comprising approximately < 1% of all uterine malignancies. Currently, malignant stromal tumors are subdivided into endometrial stromal sarcoma (ESS -previously known as low-grade endometrial stromal sarcoma) and high-grade/undifferentiated forms, which are distinguished by clinical behavior, histologic appearance, immunohistochemical features and chromosomal translocations [1,2]. CD10 was the most commonly used immunohistochemistry marker for ESS.…”