Hormonal therapy of endometrial stromal sarcoma

Abstract

Hormonal therapy with progestins, aromatase inhibitors and gonadotropin-releasing hormone analogues has become an effective treatment alternative to radiation and chemotherapy for low-grade endometrial stromal sarcoma patients. Preventive hormonal therapy is of particular interest in the setting of concomitant endometriosis.

Long-term treatment of residual or recurrent low-grade endometrial stromal sarcoma with aromatase inhibitors: A report of two cases and a review of the literature

“…Aromatase inhibitors decrease levels of estrogen by peripherally inhibiting estrogen synthesis. First-generation (aminoglutethimide) and second-generation (formestan and fadrozole) aromatase inhibitors were non-specific non-steroidal aromatase inhibitors, which exhibited serious side-effects due to the inhibition of mineralocorticoid and glucocorticoid synthesis (9). The third-generation non-steroidal aromatase inhibitors exemestane and letrozole, and the steroidal aromatase inhibitor anastrozole, exhibit minimal effects on the adrenal glands and can be orally administered.…”

“…There are several anecdotal reports of low-grade ESS that responded to aromatase inhibitors, particularly the third generation of these drugs, with more favorable toxicity profiles and thus a wider therapeutic index (6,9). However, due to the rarity of these tumors, there are no prospective or large retrospective studies on aromatase inhibitor use in the treatment of low-grade ESS.…”

“…However, progestins can cause edema, weight gain, vaginal bleeding, hypertension and thromboembolic problems (9).…”

Hormonal Treatment of Metastatic Endometrial Stromal Sarcomas

“…They make up fewer than 1% of all gynaecological malignancies. 1,2,5,6 The reported incidence is approximately one to two cases per million women in the US. 6,7 Women often present with an enlarged uterus or have complaints of heavy menstrual bleeding.…”

“…The World Health Organization (WHO) classifies endometrial sarcomas as LGESS and undifferentiated endometrial sarcoma. 5 Endometrial stromal nodule, a benign tumour and LGESS recapitulate the endometrial stroma of proliferative endometrium. How they differ is based on the growth pattern: endometrial stromal nodules are circumscribed;…”