Uterine preservation in low-grade endometrial stromal sarcoma

Abstract

Data on uterine preservation in the management of low grade endometrial stromal sarcoma (LGESS) is scarce due to rarity of this tumor type. Standard management of LGESS involves extrafascial hysterectomy with bilateral salpingo-oophorectomy with debulking of any extrauterine metastatic disease. High estrogen and progesterone receptor expression facilitates adjuvant hormone therapy post-surgery. LGESS frequently affects young women, thus fertility preservation is an important issue in management. Here we describe uterine preservation in two young women diagnosed with LGESS followed by GnRH analogue therapy with favorable outcome. The first case was diagnosed with recurrent endometrial polyp invading myometrium requiring wedge resection of uterus with free margins. Second case presented with a vaginal mass arising from cervix and excision was done through vaginal route. Both patients were prescribed GnRH analogue therapy for six months post-surgery and are currently on follow-up. These case reports add to literature on feasibility of uterine preservation in the management of LGESS.

Keywords: Fertility preserving surgery; Hormonal therapy; Low grade endometrial stromal sarcoma; Uterine preservation.

Fig. 1

1A: Plain T2 weighted MRI image in sagittal section showing well defined T2 hyperintense homogenously enhancing lesion in subendometrial region of fundus of uterus. 1B: Contrast enhanced T1 weighted MRI image in sagittal section showing heterogeneously enhancing solid lesion with multiple T1 hypointense internal cystic spaces, with T1 hyperintense hemorrhagic components within, arising from upper 2/3rd of vagina with no extension to adjacent organs 1C: 8 x 8 cm fleshy vaginal mass arising from the anterior lip of cervix.

Fig. 2

Hematoxylin and eosin (H&E) stained section shows oval to spindle shaped cells arranged in islands and sheets(x200) (A), Higher magnification shows oval to spindle shaped cells along with intervening blood-filled capillaries with no features of high-grade atypia (x400) (B). Immunohistochemistry for Estrogen receptor (ER) shows focal positivity (C), Progesterone receptor (PR) shows diffuse positivity (D), BCL6 transcriptional corepressor (BCOR) shows negativity (E), and shows ki67 labelling index of 10–12% (F).

Fig. 3

Haematoxylin and eosin (H&E) stained section shows irregular, densely cellular islands of tumor cells invading the myometrium (x40) (A). Hypocellular areas displaying focal whirling of tumor cells around arteriolar-type vessels, similar to proliferative-phase endometrial stroma (x100) (B). Higher magnification shows tumor comprising of monotonous spindle to oval cells with minimal nuclear atypia in a background of collagenous stroma (x200) (C). These tumor cells are diffusely immunopositive for CD10 (D), Estrogen receptor (ER) (E) and Progesterone receptor (PR) (F). The tumor cells are focally immunopositive for Cyclin D1 (G) and BCL6 transcriptional corepressor (BCOR) (H).