ObjectivesThe aim of this study was to evaluate the factors associated with progress-free survival (PFS) and overall survival (OS) in patients with high-grade endometrial stromal sarcoma (HG-ESS).Patients and methodsA total of 40 patients were enrolled in this study at the Peking Union Medical College Hospital, Beijing, China, from 2006 to 2016. The study retrospectively analyzed clinical and pathological data, and associations of these variables with PFS and OS were evaluated.ResultsThe age of the patients at the time of diagnosis ranged from 16 to 73 years. Abnormal vaginal bleeding was the most commonly observed symptom. The tumor size ranged from 2 to 19 cm. The tumor locations were as follows: vulva (1 case), ovary (2 cases), broad ligament (2 cases), cervix (7 cases), and uterus (28 cases). A total of 34 (85%) and 6 (15%) patients underwent complete and ovarian preservation surgery, respectively. Notably, 33 (82.5%), 13 (32.5%), and 5 (12.5%) patients received adjuvant chemotherapy, radiation therapy, and hormone treatment, respectively. Lymph node dissection was performed in 15 (37.5%) patients (positive rate: 7.4%), 16 (40%) patients underwent omentectomy (positive rate: 10%), and 12 (30%) patients underwent peritoneal lavage cytology (positive rate: 0%). Eighteen (45%) patients had lymphovascular space invasion, 13 (32.5%) patients had uterine fibroids, and 11 (27.5%) patients were diagnosed with endometriosis. Moreover, the levels of CA125 in the serum were measured prior to and following treatment. The median PFS and OS were 9 and 24 months, respectively. Eventually, 29 (72.5%) patients experienced relapse and 19 (47.5%) patients expired due to the disease.ConclusionPatients with advanced HG-ESS (stage II–IV) were associated with poor prognosis. The minimum value of CA125 and endometriosis were independent risk factors for PFS. The stage of disease, size of the tumor, minimum and average values of CA125, menopause, history of uterine leiomyoma, and endometriosis were independent risk factors for OS. The combination of surgery with radiotherapy and chemotherapy may improve the PFS of patients in the early stage of the disease.