Introduction/Background Endometrial stromal sarcomas (ESS) are very rare tumors accounting for less than 1% of all uterine cancers. The optimal therapy and outcomes are not well defined. The aim of this study was to evaluate the clinical presentation and the outcomes of ESS.

Methodology A retrospective analysis was performed involving 20 patients with histologic-proven ESS treated at Salah Azaiez Institute between 2000 and 2022. Survival outcomes were evaluated by using Kaplan-Meier survival analysis and Cox proportional hazards regression models.

Results Twenty women were included. The mean age was 47 years. The average delay of consultation was 5 months. The FIGO distribution was stage I in 9 patients, stage II in one patient, stage III in one patient and stage IV in 9 patients. Fifteen (75%) patients had low-grade ESS, and five (25%) had high-grade tumors. Ten patients with low grade ESS had early stage disease (stage I/II) and 5 were stage III/IV disease. Patients with high-grade ESS were all stage IV. Tumor cells were positive for CD10 in 4 patients, Vimentin in 3 patients and hormone receptor-positive in 3 patients. Nineteen (95%) from the cohort underwent surgery with hysterectomy, bilateral adnexectomy and lymphadenectomy in 18 patients. Ten patients (50%) received adjuvant radiation therapy. Two patients received hormonal therapy. During follow-up, 2 patients presented recurrence with distant metastases. The overall five-year survival rate was 68%. Lymphadenectomy and adjuvant radiation therapy did not affect the survival of patients. Early stage disease and low-grade tumor were associated with increased overall survival (p=0.011 and p=0.014, respectively).

Conclusion Patients with low grade ESS and early stage disease had a better prognosis. Larger prospective studies are needed to define the optimal treatment strategies.

Disclosures We have no conflicts of interest to disclose. All authors declare that they have no conflicts of interest.