A case of high-grade ovarian endometrial stromal sarcoma

Endometrial stromal sarcoma (ESS) is a rare stromal tumor, accounting for only 0.2 % of female genital tract tumors. Extrauterine involvement is extremely rare, and such extrauterine endometrial stromal sarcomas are generally termed “sarcoma-like stromal sarcomas”. The ovary represents the most common site for such occurrences, harboring stromal tumors with identical characteristics to those of endometrial stromal sarcomas. Owing to the rarity of primary ovarian ESS, there is a paucity of experience in its diagnosis and management. Herein, we report a case of high-grade ovarian endometrial stromal sarcoma, aiming to enhance gynecologists' understanding of this disease.