Abstract:

Endometrial stromal sarcoma (ESS) is a rare mesenchymal neoplasm of the uterus, which is predominantly composed of endometrial stromal cells. Primary extrauterine ESS is a special type originating from extrauterine organ without intrauterine lesions. Ovary is the most common primary site. Primary intestinal ESS is rarely described. Its clinicopathologic features have not been well characterized. We report a case of ESS arising in the small intestine in a 46-year-old woman. She was treated with cytoreductive resection surgery, followed by chemotherapy, megestrol acetate, and has been in remission for more than 77 months with disease-free. The patient′s main symptoms were epigastric pain during menstruation with radiating pain in the lower back, and she had a previous history of endometriosis. The diagnosis of ESS relies on immunohistochemical detection, and radical surgery is the first-line treatment.

Key words: Primary extrauterine endometrial stromal sarcoma, Sarcoma, endometrial stromal, Intestine, small, Case reports