ISSN: 2578-4811
Authors: Anubha B*
Endometrial stromal sarcoma manifests as an infrequently discerned, malignant, high grade uterine mesenchymal neoplasm originating from endometrial stroma and composed of spherical or spindle shaped cells. Additionally designated as undifferentiated endometrial sarcoma, uniform subtype or high grade endometrial stromal sarcoma with YWHAE-FAM22 genetic fusion; the cellular tumefaction may occasionally be associated with a low grade component. Contingent to specific molecular alterations, tumour cells appear variably immune reactive to BCOR, cyclin D1 or CD10. Smooth muscle markers and hormone receptors may be focally immune reactive or nonreactive. Endometrial stromal sarcoma manifests subtypes as ~sarcoma demonstrating YWHAE-NUTM2A/NUTM2B genetic fusion ~BCOR rearranged endometrial stromal sarcoma ~endometrial stromal sarcoma exemplifying BCOR internal tandem duplication (ITD). Exceptionally encountered, endometrial stromal sarcoma manifests with wide age range of disease emergence. YWHAE-NUTM2A / NUTM2B genetic fusion may appear within third decade to seventh decade with mean age of disease emergence at 50 years. BCOR rearranged endometrial stromal sarcoma emerges within third decade to seventh decade with median age of disease occurrence at 54 years. Endometrial stromal sarcoma with BCOR ITD emerges within second decade to sixth decade with median age of disease occurrence at 42 years [1,2]. Commonly, endometrial stromal sarcoma undergoes high grade metamorphosis within fifth decade to eighth decade with median age of disease occurrence at 54 years. Endometrial stromal sarcoma commonly incriminates uterine corpus. The vagina is infrequently implicated [1,2]. Of obscure aetiology, endometrial stromal sarcoma exhibits numerous genetic alterations denominated as YWHAENUTM2A/ NUTM2B genetic fusion, BCOR genetic fusion or BCOR ITD.
Keywords: Endometrial Stromal Sarcoma; Monomorphic; Pseudo Papillary; Pleomorphism; Myxoid Stroma; Eosinophilic Cytoplasm; Atypical Nuclei
