A Primary Low-Grade Vaginal Extrauterine Endometrial Stromal Sarcoma: A Case Report.

To analyze the clinical presentation, the diagnostic and therapeutic outcomes of a primary vaginal Low-grade EESS. Managing an LG EESS, such as VESS, is a complex task requiring multi-disciplinary team's involvement. This is crucial to ensure good prognosis due to the non-specific clinical presentations of EESS. A low-grade EESS, such as VESS, requires a multi-disciplinary team to ensure good prognosis. Primary LG-extra-uterine endometrial stromal sarcoma is a rare mesenchymal tumor that can occur at any extra-pelvic site with high recurring rate; therefore, adjuvant therapy such as aromatase inhibitors post-operative is recommended.