• Ningxin Chen, Min Gong, Wen Lai, Ling Ling, and Qiaoling Liu.
• Department of Obstetrics and Gynecology, Nanjing Medical University, Nanjing, Jiangsu, China.
• Medicine (Baltimore). 2023 Mar 24; 102 (12): e33306e33306.

RationaleEndometrial stromal sarcoma (ESS) is a rare disease in patients with uterine malignancies, accounting for <1%. Low-grade endometrial stromal sarcoma (LGESS) accounts merely 0.2% of gynecologic malignant tumor. Primary low-grade extrauterine endometrioid stromal sarcomas (LGEESS) is even more uncommon, with only a few documented case reports. We report a case of primary LGEESS exhibiting widely invasion in multiple organs after hysterectomy, which is the first case reported in Jiangsu Province of China.Patient ConcernsA 42-year-old nulliparous female with dysgnosia presented with a moderate amount of irregular vaginal bleeding, abdominal pain and distension, and frequent urination for 2 days. Her surgical history included a total hysterectomy and bilateral salpingectomy for uterine fibroids 6 years ago. Ultrasonography and the abdominal and pelvic computed tomography scan detected some solid polycystic masses in the pelvic and abdominal cavities.DiagnosesThe histopathology of the specimen confirmed the diagnosis of LESS in the absence of florid endometriosis. The patient was diagnosed with primary extrauterine endometrial stromal sarcoma at FIGO stage III.InterventionsSurgery and histopathology were performed.OutcomeAfter surgery, the patient was maintained on leuprorelin acetate microspheres with sustained release for injection at 3.75 mg once every 4 weeks while refusing further radiotherapy.LessonsThe diagnosis of primary LGEESS is challenging mainly because of their unforeseen location and nongynecologic signs and symptoms. Total hysterectomy and bilateral salpingo-oophorectomy are recommended to LGESS, while additional resection for extrauterine disease depends on disease extent and resectability.Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.

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